A female baby was born at 37 weeks and 6 days gestation by vaginal delivery with omphalocele, exstrophy of the cloaca, and imperforate anus, indicating the presence of OEIS complex, a rare combination of defects consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S), associated with lumbosacral lipoma. The most common associated spinal deformity is terminal myelocystocele, and spinal lipoma is rare. Constructive interference in steady-state magnetic resonance imaging clearly revealed double lipomas, a dorsal-type lipoma, located dorsal to the low-lying conus medullaris, and a filar-type lipoma, revealed by a thickened and fatty filum terminale. After recovery from abdominogenital repairs, debulking of the dorsal-type lipoma and untethering of the spinal cord by sectioning of the filar-type lipoma were performed at the age of 14 months. Neurosurgical treatment for occult spinal dysraphism should be undertaken after recovery from the initial series of major abdominogenital procedures.