Coccygeal polypoid eccrine nevi (CPEN) are rare, benign, cutaneous polypoid lesions localized to the coccyx region that are characterized by areas of hyperplastic eccrine ducts without hyperhidrosis. We present the case of an asymptomatic 16-month-old female with a congenital lesion in the lower sacral area and review the literature and the differential diagnosis for CPEN.