Unilateral external oculomotor nerve palsy and nevus sebaceous of Jadassohn.

A patient is presented with a congenital, external, unilateral oculomotor nerve palsy associated with the nevus sebaceous of Jadassohn. The nevus sebaceous of Jadassohn frequently has malignant potential and can be the presenting manifestation of a neurocutaneous syndrome. The ocular abnormalities of the syndrome are reviewed. Attention is called to features which overlap those of the oculoauriculovertebral dysplasia of Goldenhar.

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