Malignant melanoma of the optic nerve head in a case of oculodermal melanocytosis

Malignant melanoma of the uveal tract, orbit, and brain have been reported to occur in patients with oculodermal melanocytosis. A 60-year-old Caucasian man with oculodermal melanocytosis developed a malignant melanoma of the optic nerve head in the left eye. This case is the first reported example of a malignant melanoma developing in the optic nerve associated with oculodermal melanocytosis. After presentation the patient refused surgery for 19 months and the progression of the tumour necessitated an exenteration of the orbit.