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