In type 2, loss of heterozygosity leads to homo- or hemizygosity, with a pronounced segmental manifestation of lesions in the affected segment.[1, 2] Though the exact molecular etiopathogenesis of multiple cutaneous leiomyomas is not known, recent studies have demonstrated the involvement of a classical tumor suppressor gene encoding fumarate hydratase,[3, 4] in the pathogenesis of multiple leiomyomas. Here, FH is linked to leiomyoma cutis.