Nervonic acid (NA) has attracted considerable attention because of its close relationship with brain development. Sources of NA include oil crop seeds, oil-producing microalgae, and other microorganisms. Transgenic technology has also been applied to improve the sources and production of NA. NA can be separated and purified by urea adduction fractionation, molecular distillation, and crystallization. Studies on NA functionality involved treatments for demyelinating diseases and acquired immunodeficiency syndrome, as well as prediction of mortality due to cardiovascular diseases and chronic kidney disease. This mini review focuses on the sources, production, and biological functions of NA and provides prospective trends in the investigation of NA.