Search of the literature thus far indicates no prior description of rhinophyma occuring in tuberous sclerosis. There have been numerous reports of the usual skin lesion, adenoma sebaceum, together with the associated manifestations of severe mental retardation and convulsive seizures. In a 27-year-old woman, full-blown, severely deforming rhinophyma had its onset one year prior to her hospital admission. The family, horrified by her appearance, desired surgical intervention. The report includes a review of the literature and a description of the patient and of the surgical technique employed. The question of the desirability and difficulty involved in skin grafting is discussed, as are the microscopic findings and postoperative course. Particular attention is directed toward differential diagnosis and associated findings.