The clinical course of 42 children with lipomatous lesions of the lower spine is reviewed. Good neurological exam, IVP and myelography are considered essential components of the preoperative studies. The surgical aim is to free all intra- to extradural adhesions with dural grafting, if necessary. The results suggest that early corrective surgery within the first few weeks to months of life is the most satisfactory approach to preserving normal neurological and urological function in these children. 88% of children not operated on early developed progressive loss of neurological function and while delayed surgery frequently stopped progression of the disease it rarely returned the children to a normal state.