Background: Presence of heterotopic dysplastic renal tissue in the lumbosacral region is an extremely uncommon condition.
Case report: We report the first case of diastematomyelia associated with ectopic renal tissue. A 10-month-old male child presented with lipomeningomyelocele associated with spina bifida in the lumbosacral region, and the lipoma was excised. Imaging of the spine at 5 years of age showed spina bifida, bony diastematomyelia, lipomeningomyelocele and a small intraspinal cystic lesion. The boy was then operated upon at the age of 5 years, and histopathological examination of the cystic lesion revealed ectopic dysplastic renal tissue.