A frameshift deletion in exon 4 of FH gene (c.413-414del, p.Lue138fs) was identified in three family members (H7, H8, H9) and was associated with early onset RCC (11, 19 and 43 years), cutaneous leiomyoma, and uterine leiomyoma in all affected family members, with one additionally experiencing cutaneous leiomyosarcoma. Here, FH is linked to cutaneous leiomyosarcoma.