Basal cell carcinoma of the buttock is rare, and only 28 patients have been described. An extensive literature search was performed on basal cell carcinoma and buttock using the PubMed database. The literature was evaluated, and the characteristics of individuals with basal cell carcinoma of the buttock were summarized. The calculated prevalence of basal cell carcinoma of the buttock was 0.35%. The majority of patients had no obvious basal cell carcinoma-associated risk factors. Carcinomas were observed 1.2 times more often in women than in men, and more often on the right buttock. They frequently presented as an asymptomatic nodule. The buttock tumor was typically associated with a nodular histologic subtype of basal cell carcinoma. The prognosis for these patients was excellent after complete removal of the tumor. In conclusion, basal cell carcinoma of the buttock is a rare clinical variant of this type of skin cancer that usually presented as an asymptomatic nodule in an individual who did not have any traditional risk factors for this malignancy. The cancer was typically associated with a nonaggressive histologic subtype. Recurrence or metastasis was not observed after treatment of the primary tumor.