Myelin is a highly specialized membrane structure, wrapping around the axons. It is essential for the protection of axons, insulation and maintenance of the saltatory conduction of the action potential. Myelin membrane is rich in lipids, however, the lipid composition varies significantly from other biological membranes. Since myelination requires extraordinarily high level of lipid synthesis, the integrity of myelin is susceptible to numerous lipid metabolism disorders. Studies on transgenic mice targeting key molecules of various lipid biosynthesis pathways have elucidated the lipid metabolism and functions of myelin. Besides, myelinating glial cells have a remarkable capacity to take up extracellular lipids, which also contributes to myelination. Therefore, understanding the metabolism and functions of myelin lipids will help us to understand the role of lipids in myelin damage-related diseases and provide novel strategies for the treatment of demyelinating diseases. In this paper, some progresses in metabolism and functions of myelin lipids are reviewed.