Manifestations in tuberous sclerosis such as seizure, mental retardation, end-stage renal disease (ESRD), and heart problems present a number of challenges to the dentist and anesthesiologist. Lack of cooperation in addition to the severe medical condition makes dental treatment more difficult. General anesthesia is often required for mentally and physically handicapped patients undergoing extensive dental treatment. We experienced a case of dental treatment under general anesthesia in a 36-year-old male patient with tuberous sclerosis associated with ESRD and mental retardation. He was treated on an outpatient basis followed by hemodialysis without any complications.