Lipomas of the spinal cord are among the most fascinating lesions encountered by the pediatric neurosurgeon. An understanding of spinal lipomas may, however, be difficult because the terminology used to describe the accumulations of spinal fat is confusing, inconsistently applied, and at times contradictory. An anatomical characterization of lipomas may assist in understanding these lesions. Lipomas of the spinal cord are very rare and cause symptoms related to mass effect and secondary compressive myelopathy. Lipomas of the conus medullaris (or lipomyelomeningocele) are the most common form of fatty masses in the spine and can be divided into dorsal, caudal, and transitional forms. These lesions are a manifestation of occult spinal dysraphism and a common cause of the tethered cord syndrome (TCS). The natural history of untreated lipomyelomeningocele, although incompletely understood, appears to be progressive neurological deterioration with loss of bladder control. Timely, careful surgical intervention may prevent significant neurological deterioration and progressive disability in the majority of children harboring these lesions. In surgical intervention the surgeon seeks to disrupt the connection between the fibrofatty mass and underlying cord as well as to reestablish normal anatomical planes. Several intraoperative video segments illustrating lipomyelomeningocele resection are included in this paper. Lipomas of the terminal filum (fatty filum) are truly occult and are also associated with TCS. Surgical treatment of filum lipomas carries significantly lower risk than that for lipomas of the conus medullaris. Again, the goal of surgery is to disrupt the connection between the abnormal fibrofatty tissue and the underlying spinal cord.