The medical history of a patient with a familial atypical multiple more melanoma syndrome revealed a combination of rare findings: occurrence of a melanoma in both eyes, development of 3 separate skin melanomas, and occurrence of other malignancies in the family. Another patient developed a choroidal and a skin melanoma, but there were no known malignancies in the family. A third patient with a choroidal melanoma had a mother with an ethmoidal melanoma.