The encephalocraniocutaneous lipomatosis (ECCL), also known as Fishman or Haberland syndrome, is a rare neurocutaneous syndrome of unknown etiology. Clinically characterized by skin, eye and central nervous system lesions. We present the case of a 7-year-old female who presents to the outpatient clinic of Pediatric Neurology because of the presence of seizures detecting clinical and neuroimaging manifestations compatible with ECCL. The objective of this article is to present the case because of its rare presentation and the variety of alterations found in the tomography.
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