We report the case of a 43-year-old woman with basal cell carcinoma (BCC) of the umbilicus. Although BCC is a common skin tumor, only 2 cases of BCC arising within the umbilicus have been reported previously. Our review of the literature shows that truncal BCCs frequently develop in younger patients, often grow larger, and are associated with an increased risk for developing multiple nonmelanoma skin cancers. Therefore, we advocate a low threshold for performing biopsies on umbilical lesions that are atypical in appearance, course, or response to therapy.