Malignant melanoma oftheoptic nerveheadina caseofoculodermal melanocytosis

melanoma oftheuveal tract, orbit, andbrainhavebeenreported tooccurin patients withoculodermal melanocytosis. A 60-year-old Caucasian manwithoculodermal melanocytosis developeda malignant melanoma oftheoptic nerveheadintheleft eye. Thiscaseisthefirst reported example ofa malignant melanoma developing intheoptic nerve associated with oculodermal melanocytosis. After presentation thepatient refused surgery for19monthsandthepro- gression of thetumournecessitated an exenteration oftheorbit.