Vitiligo is a common skin disorder characterised by the presence of depigmented macules resulting from the destruction of cutaneous melanocytes. Autoimmunity is an important hypothesis with regard to vitiligo aetiology and pathogenesis and, in this review, the evidence for autoimmune responses being involved in the development of vitiligo will be discussed. Particularly relevant are autoantibodies and autoreactive T cells in vitiligo patients that have cytotoxic effects upon pigment cells. Furthermore, predisposition to vitiligo appears to be associated with certain alleles of the major histocompatibility complex class II antigens as well as with genetic variants in other autoimmune-susceptibility genes. Moreover, the association of vitiligo with autoimmune disorders, animal models of the disease, and the positive response to immunosuppressive therapeutic agents emphasise the potential role of autoimmunity in the development of this disorder.