Congenital lipomatous malformations of spinal cord constitute a diverse group of lesions and lipomyelocele is one of them. Here, we report a case of congenital lipomyelocele in a male child who presented at 7 years of age. Magnetic resonance imaging (MRI) revealed a lesion in lumbosacral region. The patient was operated and histologic examination diagnosed the case to be lipomyelocele. But after 8 years, recurrence of the lesion occurred and MRI revealed the lesion at the same location. This time histologic examination detected another new component (schwannomatous areas) in the lesion. The child was on 1-year follow-up which was uneventful.