Three infants had linear sebaceous nevus syndrome. It is suggested that this syndrome be considered a hamartoma variant. In the first patient, postmortem examination of the kidneys revealed bilateral nodular nephroblastomatosis. Cortical blindness and intractable seizures were noted in the second patient. Cardiac involvement was present in two patients. Similarities between linear sebaceous nevus syndrome and tuberous sclerosis include involvement of numerous organ systems in addition to the skin and brain.