We present a female baby with a human tail associated with congenital dermal sinus (CDS) at the caudal site of the tail. Magnetic resonance (MR) images with constructive interference in steady-state (CISS) sequencing clearly demonstrated a lumbosacral lipoma of caudal type, contiguous with the dermal sinus tract and not with the human tail. At 3 months old, the tail was surgically removed. The dermal sinus tract and contiguous lipoma were also resected, and untethering of the spinal cord was performed. Although both the human tail and CDS are frequently associated with spina bifida occulta, coexistence of the tail and CDS is exceedingly rare. CISS-MRI is useful for demonstrating the precise anatomical relationship between these complicated pathologies.
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