facial angiofibromas seen in tuberous sclerosis are usually bilateral.unilateral facial angiofibromas are seen rarely . unilateral facial angiofibromas , however , represent a rare variant of tsc , as only few cases are reported in the literature . we describe a case of a 30-year - old male who presented with unilateral facial angiofibromas without other features of tsc . a-30-year - old unmarried male born of non - consanguinous marriage presented for evaluation of right - sided facial papules that were first noted at 15 years ago . the lesions reportedly began as flat - topped , skin - colored , and erythematous papules involving the right side of his face . he denied history of any previous treatment . during last five years , gradually increasing numbers of new and similar lesions have appeared and spread to involve the right cheek , right nasofacial sulcus , and the right side of the nose . on examination , the patient has multiple , discrete , 2 - 5 mm , flat , skin - colored and hyper - pigmented papules involving the right cheek and right side of the nose [ figure 1 ] . there were no periungual fibromas , shagreen patches , caf - au - lait macules , forehead plaques or hypopigmented patches . the patient has no evidence of mental retardation and no history of seizures , cardiopulmonary lesions , renal lesions , or retinal abnormalities . multiple skins colored to hyperpigmented papules of various size distributed unilaterally on right side of face our clinical differential diagnosis included angiofibromas , verruca plana , syringoma and trichoepitheliomas . as there were no other cutaneous signs of tsc , the patient denied further evaluation and imaging studies to rule out internal manifestations of tsc . skin biopsy specimens of the facial papules revealed characteristic findings of angiofibromas , namely telangiectases , perivascular and periadnexal concentric dermal fibrosis , and large stellate fibroblastic cells scattered in the upper dermis . [ figures 2 and 3 ] based on above observations a final diagnosis of unilateral facial angiofibromas was made . patient was advised cryotherapy for his lesions , but we lost the follow - up with patient . ( h and e , 50 ) high power view showing dense collagen thickening , dilated vessels and few melanophages ( h and e , 200 ) the major impact of the disorder is on the skin , brain , and kidneys , though it also affects the eyes and heart . a major point regarding symptoms and signs of tsc is the variable clinical expression of the disorder . the major manifestations of tsc include skin lesions in more than 95% , mental retardation in approximately 50% , autism , seizures in approximately 85% . the classic triad of mental retardation , seizures and angiofibromas occur in only 29% of patients and 6% of them lack all three manifestations . facial angiofibromas occur in 80% to 90% of patients with tsc , typically presenting after 5 years of age . they typically occur as bilateral and symmetric , small , tan to erythematous papules on the cheeks , chin , and nose . our patient has multiple angiofibromas affecting only one side of the face but no other characteristic features of tsc were present , which is a rare presentation of tsc . mcgrae and hashimoto reported a case of unilateral facial angiofibromas as a segmental form of tsc . in 1997 , anliker , et al . and in 1998 , garcia , et al . reported similar cases . in 2000 , silvestre , et al . reported two patients with similar findings as a mosaic form of tsc . trauner , et al . in 2003 , reported similar case with poliosis of occipital area in a 53-year old man . in 2006 , camprubi , et al . reported a similar case in 13-year - old male and reviewed previous cases . hall , et al . in 2007 reported a case similar to our present case without any other manifestations of tsc . in 2008 , bordel - gmez reported a 28-year - old male with unilateral angiofibromas . lopez , et al . in 2009 , reported a case of unilateral eyelid angiofibroma with complete blepharoptosis as a presenting sign of tsc . our case of unilateral facial angiofibromas and without any other manifestation represents a rare clinical presentation of tsc . however , the possibility that this case represents a distinct clinical disorder can not be ruled out completely . this case highlights a rare occurrence of unilateral multiple facial angiofibromas without any other manifestations of tuberous sclerosis .