Tuberous sclerosis (TSC) is an autosomal dominant inherited disorder with cutaneous lesions of ash leaf hypopigmented macules, shagreen patches, periungual fibromas, facial angiofibromas, forehead fibrous plaques, confetti hypopigmentation, and poliosis. Multiple facial angiofibromas are a pathognomonic feature of TSC. Unilateral facial angiofibromas, however, represent a rare variant of TSC, as only 6 cases are reported in the literature. We describe a case of a 52-year-old man who presented with unilateral facial angiofibromas and poliosis without other features of TSC.