Perinevoid alopecia (PA) is a rare variant of alopecia areata (AA) associated with a central pigmented nevus. In this study, we reported two cases of PA and reviewed 14 cases from 11 studies in the literature. In one of our cases, PA was combined with a halo nevus and white terminal hairs were spared in the hair loss patch, which was rarely reported in the literature. It is implicated that antigens from melanocytes might be involved in the development of AA in PA.