a 16-year - old female patient came to department of oral medicine and radiology with chief complaints of bleeding from gums . on examination , she was thin built . extraoral examination of face and skull revealed hypoplasia of the malar eminences and zygomata , hypoplasia of maxilla and underdevelopment of the mandible [ fig . 1 ] . ears appeared low - set and rotated with presence of skin tags [ fig . 2 ] . intraoral examination revealed inflamed gingiva due to poor oral hygiene . radiological examination supported the clinical findings . patient was then referred to ophthalmology department where ocular examination showed downward slant of palpebral fissures , ptosis of upper lids and limbal dermoid in right eye measuring around 1  1 cm in diameter [ fig . the patient was uncooperative and refused any other treatment except for oral prophylaxis which we performed to relieve her from bleeding gums . extraoral examination of face and skull showing hypoplasia of the malar eminences and zygomata , hypoplasia of maxilla and underdevelopment of the mandible low - set ears along with presence of skin tags ocular examination showing limbal dermoid in right eye the most ordinary type of acrofacial dysostosis is the nager type ( nafd ) of the so - called pre - axial acrofacial dysostosis . this disorder is categorized by the office of rare diseases in the united states as a rare illness ; thus , it has only affected less than 200,000 of the country 's population . some studies have shown that the gene which causes the illness might be located around 9q32 and it is possible that the zfp-37 gene could also be involved . the major symptoms of nager syndrome often include the underdevelopment of the jaw and cheek areas , a jaw that may appear smaller than the usual or normal jaw , hearing problems ( and some defects on the external or internal ear area ) , cleft palate ( which is a congenital defect of the mouth or the lip ) , the absence or underdevelopment of the thumb or thumbs , forearms that appear short ( the forearms could altogether be absent of the radius bone ) , fusing of the bones of the forearms , poor elbow movements ( or limited extension of this body part ) , ears that appear to be backward rotated , the ears manifesting some skin tags ( or they could be low - set ) , and feeding and breathing problems ( especially among infants who are afflicted with this syndrome ) . our patient showed underdevelopment of jaws , hypoplasia of the malar eminences , zygomata and hypoplasia of maxilla . ocular manifestations of this syndrome are rare and include downward slant of palpebral fissures , ptosis of upper lids , coloboma of lower lids , and deficiency of eyelashes of the medial one - third of the lower eyelids . our patient showed downward slant of palpebral fissures , ptosis of upper lids and limbal dermoid in right eye . traditionally , the differential diagnosis of nager syndrome is done with treacher - collins , pierre - robin and goldenhar syndrome . treacher - collins syndrome is characterized by maxilla and mandible hypoplasia , downslanting palpebral fissures , colobomas of inferior eyelid and auricular malformation . pierre - robin syndrome presents with micrognathia , cleft palate and glossoptosis . in goldenhar syndrome , we find hemifacial microsomy , flattening of maxilla , temporal and zygomatic bone , microtia , ocular and vertebral alterations , and in 50% of cases , fallot tetrad . the echographic findings of these malformations become evident usually in the third trimester , therefore a systematic ultrasound exploration including visualization of fetal face line is essential for its detection . if the patient suffers from speech retardation , then a speech therapist could help the patient . they may not totally cure the condition , but they can help in managing the symptoms . treatment of limbal dermoids may consist of periodic removal of irritating cilia , topical lubrication to prevent foreign body sensation , or excision of the lesion if it is causing significant cosmetic disfigurement or interfering with vision . our patient refused to take any ocular treatment and insisted on taking only the dental treatment for which she visited our hospital . correct understanding of the underlying pathophysiologic pattern of this disease is necessary for optimal diagnosis and treatment planning . ophthalmological examination of these patients is a must as ocular manifestations are not uncommon in these patients .