Neuromyelitis optica spectrum disorders (NMOSD) is an autoimmune demyelinating disease in the central nervous system. There is increasing evidence that lipid metabolism is associated with adverse clinical disease activity in NMOSD. In this review, we summarized the correlation between low density lipoprotein (LDL), high density lipoprotein (HDL), triglyceride (TG) and NMOSD activity. Although the causal relationship between disease activity in NMOSD and abnormal lipid metabolism has not been elucidated, we believe that advances in this area of research have the potential to improve understanding of the pathophysiology of neuromyelitis optica and identify new treatment targets and strategies.