Melanomas are rare in patients who have albinism, compared with the frequent occurrence of squamous cell carcinomas and basal cell carcinomas. This report describes amelanotic metastatic melanoma in a 58-year-old Japanese man who had tyrosinase-positive oculocutaneous albinism. A prolonged bleeding time, facioscapulohumeral muscular dystrophy, and Gilbert syndrome were also present. Superficial spreading melanoma with evidence of spontaneous regression on his right forearm was suspected as a possible primary site. Twenty-two cases of melanomas in persons who have albinism have been reported.