Halo nevus is a pigmented nevus surrounded by acquired round depigmentation. Depigmentation appears mostly around several types of pigmented lesions, such as acquired nevus, congenital nevus, blue nevus and Spitz nevus. In
contrast, it is rare that depigmentation develops around a congenital nevus. While the precise etiology of halo nevus and vitiligo remains uncertain, several theories suggest that both phenomena result from an immunologic reaction to melanocytes by CD8 ＋ T cell. We report a case of halo congenital nevus in the arm, which is followed by periocular vitiligo. This is the third case in Korean dermatological literature.