Encephalocraniocutaneous lipomatosis is a congenital neurocutaneous disorder with the distinguishing histopathological features of dysgenesis and neoplasia of the adipose tissue. The dominant clinical features of the syndrome include convulsions beginning in infancy, mental retardation, and unilateral cutaneous and ophthalmological lesions with ipsilateral cerebral malformations. A patient with this rare disorder of ectomesodermal dysgenesis has been studied in order to classify clinically and histologically the associated skin lesions. To our knowledge, this is the fourth case of encephalocraniocutaneous lipomatosis reported in the English literature.