This male neonate was born at 40 weeks of gestation by vaginal delivery after an uneventful pregnancy. At day of life 3, he presented with seizures characterized by episodes of apnea and cyanosis. Craniofacial dysmorphic features including a left facial nevus psiloliparus, a left choristoma and cleft eyelid, and scalp lipomas prompted the diagnosis of encephalocraniocutaneous lipomatosis. Phenobarbital and levetiracetam controlled the seizures well. At age 5 months, he developed infantile spasms. Adrenocorticotropic hormone therapy controlled infantile spasms for about a month before they recurred. The seizures remained refractory to multiple antiepileptic drugs, and the child developed Lennox-Gastaut syndrome. At age 2 years, a vagus nerve stimulator was implanted with remarkable seizure improvement. A spine magnetic resonance imaging at age 2 years showed a T1and T2-hyperintense, lobular lipoma in the posterior extra-axial space extending from the cervical to the thoracic spine with thinning of the adjacent spinal cord (Figure A,B). In addition, an intradural lipoma causing a spinal cord tethering was noted (Figure C,D). Brain magnetic resonance imaging showed a lipoma in the left Meckel cave with extension into the pterygopalatine fossa, a large posterior fossa arachnoid cyst wrapping around the left cerebellar hemisphere, and a hypervascular lipoma of the left scalp (Figure E,F).