RESUMEN
Proteus Syndrome is an extremely rare, sporadic and progressive disorder. We describe a four-month-old male baby with central nervous system manifestations in this article. A four-month-old boy was admitted into our hospital with three tonic- clonic generalized seizure attacks which started from the day before admission. Each seizure attack lasted less than 10 minutes and the baby was well between attacks. No fever was detected. On physical examination, abnormal facial features, macrocrania, a wide nasal bridge, overgrowth of the right lower limb, macrodactyly in the third left toe, epidermal nevus on the left side of the abdomen and skin vascular abnormalities were detected. His developmental status was normal. Brain Magnetic Resonance Imaging [MRI] revealed left sided hemihypertrophy, ventricle enlargement and macrocrania on the same side