This is a description of a male patient, now 8-years-old, with the syndrome described by Schimmelpennig-Feuerstein and Mims. Characteristic for this clinical picture, which is classified as a phacomatosis, are a striated and sponge-like naevus sebaceus, disorders of the central nervous system taking the form of debility and epileptic attacks, and multiple anomalies of the eyes, such as microphthalmus, coloboma of the eyelids and optic nerve with ablatio falciformis, as well as dermoids and teratomas of the conjunctiva.