Abstract
Aims: Encephalocraniocutaneous lipomatosis (ECCL) is a very rare sporadically occuring neurocutaneous syndrome with unilateral or bilateral manifestation, predominantly affecting the head. It is characterized by skin lesions (naevus psiloliparus, alopecia, subcutaneous fatty masses, nodular skin tags), ocular anomalies (mainly choristomas) und CNS anomalies (intracranial and spinal lipomas, intracranial vessel defects). About two-thirds of patients have a normal development or mild retardation only, and half of them have seizures. Jaw tumours and bone cysts may be associated.
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