Human tail is a rare congenital anomaly in which a lesion protrudes from the lumbosacrococcygeal region. We encountered a case of human tail involving an intradural lipoma and tethered cord, occurring in a 1-day -old female who presented with an 8 cm-sized tail shown by MRI to arise from the S3-4 level. The cauda equina and film terminale were entrapped by the lipoma, but there were no bony abnormalities.