Spinal ganglioneuroma occurs mostly in the thoracic spine causing various manifestations including scoliosis that can be misdiagnosed as idiopathic scoliosis. Few reports exist in the literature on the diagnosis and management of scoliosis secondary to huge ganglioneuroma and usually staged treatment is preferred. In this report, we present a 17-year-old female patient presented with back pain, lower limbs numbness, spinal deformity, and shortness of breath. Plain X-rays showed a 50° right thoracic scoliotic curve. MRI and chest CT revealed a huge extra pulmonary mass shifting the mediastinum with intra spinal extension through the left neural foramina compressing the spinal cord. Percutaneous US guided needle biopsy confirmed the diagnosis of ganglioneuroma. One-stage posterior instrumented correction of scoliosis, spinal cord decompression, and excision of the whole mass from the mediastinum and the spine through posterior approach was done for the patient with smooth postoperative recovery. Chest CT scan was done 2 years after surgery and excluded any local recurrence.