Encephalocraniocutaneous lipomatosis (ECCL) is a sporadically occurrin
g disorder that belongs to the group of neurocutaneous syndromes. Impo
rtant characteristics of the case we present are: intracranial lipomas
, a skull hamartoma, bilateral lipodermoids and jaw tumors (ossifying
fibromas and compound odontomas). We propose four minimal criteria for
the diagnosis of ECCL and review the ocular abnormalities reported to
date.