Ocular melanoma is the most common malignant tumor of the eye and accounts for 70 to 80 percent of all extracutaneous melanomas. Its biologic behavior differs from that of its cutaneous counterpart. To elucidate this, 62 patients with histologically proven melanoma of eye (58 uveal tract and 4 conjuntiva) at Roswell Park Memorial Institute from 1945 to 1977 were studied. The prominent contradistinctions from other head and neck melanomas were (1) a very high percentage of patients had either locally advanced or systemic disease at diagnosis, although the eye is the most sensitive organ; (2) regional lymph node involvement was absent even in the late stages of disease; (3) hematogenous spread involved single organs, most commonly the liver and the lung; (4) local recurrence was rare; (5) most recurrences occurred evenly in first 10 years after treatment; (6) regional resection, chemotherapy or both are advocated for distant metastases since they are confined to a single organ and are amenable to it; and (7) despite hematogenous spread and advanced disease at diagnosis, the overall prognosis of ocular melanoma is comparable to that of cutaneous melanoma.