Ganglioneuroma (GN) is a benign rare tumor that originates from neural crest cells. It shares a common histogenic family with ganglioneuroblastoma and neuroblastoma and represents the most benign and differentiated form of the group. The most common locations of the GNs are the mediastinum and retroperitoneum. We report an extremely rare case of lumbar spinal GN with conus medullaris invasion and extradural paraspinal extension. A 10-year-old girl presented with a history of worsening lower-back pain and an intermittent tingling sensation in the left leg. Neurological examination revealed reduced sensation in the left L2-L5 dermatomes. Magnetic resonance imaging revealed an intra- and extradural mass extending from the D11 to L5 vertebral body level. There was conus medullaris invasion by the tumor. After L1-L5 laminotomies, the patient underwent tumor resection. Histopathological diagnosis was immature GN. GNs occurring within the spinal column are rare and may grow to a large size. Despite the size and the common involvement of both intra- and extraspinal compartments, the prognosis is usually excellent after complete excision using microsurgical techniques.

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