There are various types of benign skin tumors in association with the complex nature of the skin. A minor part of them may be associated with visceral malignancies. This review focuses on the benign skin tumors that are described to have well-known associations with systemic malignancies or those with controversial associations. These tumors and the associated conditions are as follows: seborrheic keratosis/ Leser-Trelat sign, lentigo simplex/Peutz Jeghers and Carney syndrome, sebaceous tumors/ Muir-Torre syndrome, fibrofolliculoma- trichodiskoma/ Birt-Hogg-Dube syndrome, trichoepitheliomacylindroma-spiradenoma/ Brooke-Spiegler syndrome, leiomyoma/multiple cutaneous and uterin leiomyomatosis syndrome, glomeruloid hemangioma/ POEMS syndrome, venous malformation/ Maffucci syndrome, mucosal neuroma/ multiple endocrine neoplasia type 28, juvenile xanthogranuloma/ juvenile chronic myeloid leukemia. It is important to know the skin tumors related with visceral malignancies in part of a syndrome or in association with a sign as this may yield an earlier recognition of these malignancies.