Introduction: “Human tail” is a congenital, benign anomaly in which a protrusion in the lumbosacrococcygeal caudal region is covered with skin. Arteriovenous hemangioma is a benign vascular lesion that may be congenital or acquired. We present a case in which a human tail was present with pathological findings of arteriovenous hemangioma. Case Report: A 6-month-old girl was born with a curved tail-shaped protrusion along her waistline. The distal portion was purple and had a solid consistency; the proximal portion was of normal skin color and had a soft consistency. No other abnormality was observed in the spinal area. Microscopic examination revealed congested, proliferated vascular structures in the fibrous stroma distally located under the multilayered squamous epithelium. In the proximal area, mature lipomatous tissue was observed. The lesion was diagnosed as arteriovenous hemangioma. Conclusion: Our patient presented with the classic “human tail” appearance at an early age and in the lumbosacral region. Because of the pathological findings, surgery for the congenital anomaly had to be performed with extra caution.