An additional case of familial occurrence of malignant melanoma is presented, involving mother and son. This is the fourth case of familial occurrence of malignant melanoma in the female genitalia and the first occurring in the vagina reported in the literature. We present a review of the literature concerning the hereditary property of malignant melanoma, including a list of all 92 families and 240 patients reported. The importance of a family history of malignant melanoma for the early diagnosis of this disease in relatives at risk is stressed.