Encephalocraniocutaneous lipomatosis (Fishman syndrome): A rare neurocutaneous syndrome

To report a rare case of encephalocraniocutaneous lipomatosis (ECCL) presented with characteristic multiple organ involvement.

A 7-day-old white Iranian girl was referred with ocular, skin and brain abnormalities.

The findings of nevus psiloliparus, eyelid choristoma and intracranial lipoma were consistent with ECCL.

Since the skin and ocular manifestations can be easily observed at birth examination, pediatricians and ophthalmologists should be aware of this condition.