Since Bourneville1 first described tuberous sclerosis of the brain in 1880 and van der Hoeve2 in 1921 published the first known observations on the associated retinal tumors, other authors have reported additional cases of this rare disease. The condition is characterized by (1) retarded mental development, (2) the occurrence of epileptiform seizures and (3) the occurrence of tumors of one or more organs, usually the skin, the brain and the eye and often the liver, kidneys and heart and possibly the stomach. Variations from the typical clinical picture are not infrequent, as has been noted by Critchley and Earl.3 They stated that only one component of the triad may be found. Instances have been reported in which only certain members of a family (in some cases, only one member) had tuberous sclerosis, whereas other members of the same family exhibited only one or two of the components