Abstract： Segmental vitiligo,a subtype of vitiligo,is distributed segmentally along cutaneous nerves.Compared with other types of vitiligo,segmental vitiligo has distinctive clinical manifestations,pathogenesis and treatment.It is characterized by early onset,asymmetric skin lesions,rapid progression,and low likelihood of spreading to other sites or being accompanied by other autoimmune diseases.The etiology and pathogenesis of segmental vitiligo remain unclear,and several hypotheses have been put forward,such as the neural theory,genetic theory,immunological theory and a new three-step theory.The first-line treatment of segmental vitiligo is avoidance of causative factors,topical application of glucocorticoids,calcineurin inhibitors,vitamin D3 derivatives,and oral administration of a small dose of glucocorticoids at rapid progression stage.The second-line treatment is narrow-band ultraviolet B,excimer laser or helium-neon laser,etc.Surgery may serve as the third-line treatment when repigmentation is unsatisfying.