Skin tags are fairly common lesions usually seen in adults on the neck and in body folds. The sacrococcygeal region is an unusual location for skin tags in children and may represent a congenital malformation of the spine or an isolated skin lesion. In this review, we summarize the clinical presentation, histopathologic changes, and differential diagnosis of coccygeal polypoid eccrine nevus as a rare cause of sacrococcygeal papules in children.