A 65-year-old woman presented with a history of severe lower back pain on forward-flexion for 2 months duration. Magnetic resonance imaging revealed a high signal mass with a tail on T1-weighted images at the L3 level. A total surgical resection was performed via a posterior approach with the aid of a microscope. Histopathological examination of the tumor revealed two pathological components: lipoma and myxopapillary ependymoma. The presence of dual histological components in one spinal cord tumor is rare. There are no prior reports of both types of cells (adipose and ependymal) grown simultaneously in a single tumor of the filum terminale in the medical literature. We report a unique case of the co-existence of lipoma and myxopapillary ependymoma within the same tumor located at the filum terminale and review related literature.