A malignant triton tumour is a malignant schwannoma with rhabdomyoblas
tic differentiation, and is a very rare occurrence. We describe the ca
se of a 33-year-old man with neurofibromatosis type I who presented wi
th paraplegia. Pathological examination of an intradural, extramedulla
ry tumour excised at lumbar laminectomy demonstrated a malignant schwa
nnoma with rhabdomyoblastic differentiation. We believe this is the fi
rst documented case of a malignant triton tumour of the spine.