A 4-year-old child presenting with sudden-onset paraplegia and a sacral tum
our in association with spina bifida occulta is reported. There were no sti
gmata of spinal dysraphism at birth. Imaging studies confirmed a sacral tum
our with extradural extension up to T10 and spinal dysraphism. The histolog
ical features of the extradural and sacral components of the tumour were co
nsistent with a Wilms tumour. The differential diagnosis included a primary
sacral teratoma containing Wilms tumour elements or a primary extrarenal W
ilms tumour arising in association with a spinal dysraphism. There was no c
linical response to chemotherapy or radiotherapy.