Sphingolipids (SLs) are a class of lipids that are essential components of cell membranes. The main SLs and the metabolites include sphingomyelin, ceramide, and glycosphingolipids. They serve as signaling molecules and can regulate various cellular processes including proliferation, migration, senescence, and apoptosis that are essential for human health. SLs from marine organisms exhibit special structures and diverse physiological functions due to the extreme environment, which have attracted ever‐increasing attention recently. In this review, the contents and structures of SLs from marine sources, analytical methods, their metabolic pathways, as well as the physiological functions, especially the ability for the prevention and/or treatment of various diseases, including immunity, metabolic syndrome, neuroinflammatory, neurodegenerative diseases, and cancer, were discussed.