Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome. The unique clinical manifestations of the disease include ocular edema or hamartoma, abnormal skin on the head and neck, and corresponding hair loss on the scalp. Historical literature on ECCL consists mainly of case reports and does not present the common features of the disease. We present the characteristic manifestations of the eye and skin to provide ophthalmologists with a fuller understanding of in ECCL.

Pathological results: Two cases were corneal Sebaceous tumor (mature squamous tumor) and two cases were fibrous epithelial polyps.

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