Nine cases of congenital lumbosacral lipoma have been diagnosed and treated surgically at the Hacettepe University School of Medicine, Department of Neurosurgery during the last 16 years. Five patients were male and four female with ages ranging from 18 months to 30 years. The most frequent complaints were mass in the back (in five cases), motor weakness in the lower extremities (in five), urinary incontinence (in five), and lumbosacral hypertrichosis (in five). On neurological examination, four patients had deep tendon reflex changes, five had motor changes and four were normal. There was significant improvement in seven cases. Two had no improvement. In this report, we review nine patients retrospectively, and discuss the surgical intervention and the results achieved.