Human tails or caudal cutaneous appendages are rare congenital anomalies with lesions usually arising from the midline of the lumbosacrococcygeal area. There have been only around 70 cases reported in literature. A 4-month-old male infant born as the 1st child of a dizygotic twin was presented with a penis-shaped mass of 4-cm in the coccygeal area. The mass was entirely covered by skin without any skin defect and was connected to the midline skin of the posterior coccygeal area by a stalk. Magnetic resonance images showed fat density of the mass without any demonstrable communication with the spinal canal nor any underlying central nervous system anomalies. The patient underwent a simple surgical excision of the mass without residual effects and complications. Histopathologic examination of the mass revealed lobules of mature adipose tissue, fibromuscular soft tissue, small vessels and nerves with normal hair follicles on the skin.