An additional case of familial occurrence of malignant melanoma is presented, involving mother and son. This is the fourth case of familial occurrence of a 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.