A case of intramedullary spinal cord lipoma is presented. A one month-old male infant presented with irritability and weakness on his upper extremities. A magnetic resonance[MR] image of the cervical spine demonstrated a well defined, high signal intensity lesion on both T1 and T2-weighted MR images and suppression on the fat saturation sequence. The tumor mass extended from the foramen magnum to T2 vertebra level. Ventral displacement of the spinal cord with kinking of the cervico-medullary junction was evident on the T2-weighted sagittal image. Partial resection of the tumor mass through laminoplastic laminotomy from C1 to T2 resulted in improved motor weakness on his upper extremities.