We present a case of intramedullary spinal gangliocytoma in a 7-year-old girl who presented with scoliosis and progressive weakness of both legs. The tumour involved the whole spinal cord and medulla oblongata and was composed of inner cystic and outer solid components. On MRI, the solid portion of the lesion showed strong enhancement at the thoracolumbar level and mild enhancement at the cervical and medullary levels. Histological examination of the surgical specimen showed neoplastic ganglion cells arranged irregularly in benign normocellular glial background, which made a diagnosis of gangliocytoma.