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Key Clinical Message In conus medullaris, mature teratomas are rare. We report a case of a 40‐year‐old man who presented with urinary incontinence, low back pain, and muscle weakness. Magnetic resonance imaging revealed a mass in conus medullaris (T11–L1), further confirmed as a mature teratoma by pathological examination. We identified 63 cases of conus medullaris teratoma over the past two decades by systematically analyzing the case reports. Findings demonstrated that most cases were diagnosed in the fourth decade of life, with the majority of cases (57.6%) being male. Lower back pain, radiating pain in the extremities, hypoesthesia, and urinary dysfunction are the most common clinical presentations among patients with teratoma of conus medullaris. Mature teratoma is the dominant pathologic subtype of teratomas in this region, comprising more than 95% of cases. Our case highlights the importance of considering spinal teratoma as a differential diagnosis in patients presenting with urinary incontinence and lumbar pain.