A white family with three generations (one by history) showing the clinical picture of nevus of Ota is presented. In the two generations examined, no evidence of uveal malignant melanoma was found. This is the first such family reported in the English literature and the fifth family in the world literature. It shows the importance of a good family history in dealing with a patient with a nevus of Ota. Affected family members should be examined carefully to rule out uveal malignant melanoma until the questions surrounding its reported association with nevus of Ota can be resolved.