Spinal lipomas account for 5% of the tumors of the spinal cord, frequently present already at birth. Most commonly they are associated with forms of dysraphism, but lipomas without bony involvement are considered dysembriogenetic lesions too. Children with lipoma frequently have intact neurological functions, but may become symptomatic later on. Diagnosis is possible also in neurologically intact patients because of skin lesions or subcutaneous masses. Many surgeons suggest early surgery to prevent injury to neural structures from traction due to cord tethering; others prefer to wait for the rise of any symptom before considering surgery. However, neurological recovery after surgery is rarely observed, and, when present, is always partial; the primary goal of surgery is to stop the clinical progression through the detethering of the cord.