Abstract: Glomus tumors are benign lesions which often appear as solitary bluish nodules. They can also be multiple, and can be either acquired or congenital. Histopathologically glomus tumors are classified into three different variants: solid glomus tumor, glomangioma, and glomangiomyoma, which is the least frequent type. We report three instances of familial generalized multiple glomangiomyoma in a woman and her two children. Seven members of the family in two consecutive generations were affected, suggesting autosomal dominant inheritance. We performed a thorough clinical study, complete blood and platelet counts, stool for occult blood, karyotype, abdominal echography (which showed an asymptomatic solitary hepatic vascular lesion in one of the patients), gastrointestinal endoscopy, and skin biopsy with immunohistologic and ultrastructural studies. We believe that the terms glomangioma and glomangiomyoma actually designate the same lesion, with transitional areas from typical glomus cells to well-defined conventional smooth muscle cells. Although complete evaluation should be performed in all patients with multiple glomus tumors in order to detect possible occult systemic lesions, we only recommend treatment for symptomatic lesions. To our knowledge, this is the first report of an instance of familial multiple glomangiomyoma.