ameloblastoma is seen to have a benign appearance on histology , irrespective of its variable clinical behavior . microscopically various patterns have been described ( follicular , plexiform , acanthomatous , papilliferous - keratotic , granular cell type , desmoplastic , vascular , and dentinoameloblastoma).[13 ] majority of patients present in the fourth decade . men are affected slightly more often than female , with a particularly elevated incidence in eastern africa . more than 80% of ameloblastomas arise in the mandible ( mostly angle or ramus ) . they are either primary or secondary soft tissue tumors , the latter appearing after operations . radiologically , ameloblastomas present as unilocular or multilocular translucencies . malignancy in the ameloblastoma has been divided into two distant lesions . a malignant ( metastasizing ) ameloblastoma is diagnosed when a seemingly histologically benign ameloblastoma produces a metastasis resembling the original lesion . ameloblastic carcinoma is an odontogenic tumor having the overall microscopic architectural features of ameloblastoma but in addition having malignant cytological features such as marked nuclear atypia and numerous mitotic figures . in the year 1965 , tsukada et al . two years later , a case of granular cell ameloblastoma with metastasis to cervical vertebrae was reported . we reviewed the literature for any cases of granular cell ameloblastoma with metastasis , which might have been reported from year 1967 until now . to the best of our knowledge a 40-year - old female first reported to the surgery department of safdarjung hospital , new delhi , india . she presented with a disfiguring swelling on the right side of neck of approximately two years duration [ figure 1 ] . roentgenogram of the mandible showed swollen translucent cystic structures reaching from corpus to ramus of the right mandible . excision of zygomatic arch and surrounding soft tissue , masseter muscle , and temporalis muscle along with right parotid , was done . reconstruction was performed using right pectoralis major myofacial flap for buccal mucosal reconstruction and delto pectoral flap with split skin graft for skin defect . two years following surgery , patient 's follow up with x - rays , have not revealed any new lesions . patient with a large abnormal swelling on the right side of face gross examination of the excised specimen showed a well circumscribed large pink tan growth measuring 7.5  5  4 cm in the region of the mandible . tumor cells formed nests supported by fibrous connective tissue stroma [ figure 2 ] . at the periphery of the nests , the inner cell mass showed round to polygonal cells with abundant granular cytoplasm and small pyknotic nuclei [ figure 4 ] . one lymph node showed tumor metastasis characterized by the presence of granular cells [ figure 5 ] . ( h and e , 200 ) cells displaying cytoplasmic granularity with pyknotic nuclei , some of which are eccentric . ( h and e , 400 ) metastasis in lymph node showing tumor cells with granular cytoplasm . gross examination of the excised specimen showed a well circumscribed large pink tan growth measuring 7.5  5  4 cm in the region of the mandible . tumor cells formed nests supported by fibrous connective tissue stroma [ figure 2 ] . at the periphery of the nests , the inner cell mass showed round to polygonal cells with abundant granular cytoplasm and small pyknotic nuclei [ figure 4 ] . one lymph node showed tumor metastasis characterized by the presence of granular cells [ figure 5 ] . ( h and e , 200 ) cells displaying cytoplasmic granularity with pyknotic nuclei , some of which are eccentric . ( h and e , 400 ) metastasis in lymph node showing tumor cells with granular cytoplasm . the tumor is made up of proliferating odontogenic epithelium especially of enamel organ - type tissue that has not undergone differentiation to the point of hard tissue formation . it has been postulated that the epithelium of origin is derived from one of the following sources : ( 1 ) epithelial lining of odontogenic cyst , ( 2 ) dental lamina or enamel organ , ( 3 ) disturbances of developing enamel organ , ( 4 ) basal cells of surface epithelium , or ( 5 ) heterotopic epithelium of other parts of the body . our patient presented with a metastasis of granular cell ameloblastoma at the first instance , giving a brief history of 2 years . most common sites of metastasis are lung ( 76.7% ) , followed by regional lymphnodes ( 37.8% ) , pleura ( 16.2% ) , vertebrae ( 13.5% ) , skull ( 10.8% ) , diaphragm ( 8.1% ) , liver and parotid ( 5.4% ) and even more rarely , the spleen and kidney . granular cells of granular cell ameloblastoma are clearly of epithelial origin staining exclusively for cytokeratin . the term  granular cell ameloblastic fibroma  is a misnomer , as a number of these cases are probably central odontogenic fibromas exhibiting granular cell change . ultrastructurally it has been revealed that it is the lysosomal overload in these cells that imparts the characteristic granularity . whether granular cell change in ameloblastoma is a degenerative process or a harbinger of a more aggressive course is a matter of debate . all three cases of granular cell ameloblastoma ( our case included ) have eventually produced metastasis . add to this , our patient gave a short history of duration before tumor metastasis . although the exact implication of a granular cell change in ameloblastoma can not be ascertained due to paucity of cases documented , yet reported cases must be followed closely in anticipation of metastasis .