Congenital ocular melanocytosis

Congenital oculodermal melanocytosis represents a unilateral accumulation of melanocytes in the episclera, sclera and uveal tract. Clinically, it manifests with iris heterochromia, episcleral and scleral pigmented patches, dark pigmentation of the trabecular meshwork and the fundus. This congenital disorder is a predisposing factor for uveal melanoma and chronic glaucoma. We report here the case of a Caucasian woman who presents some of these characteristics.