Nevoid hyperkeratosis of the nipple and/or areola is a rare and idiopathic disorder with fewer than 50 cases reported in the literature. We report two cases, discuss the clinical and histological findings, and review the literature pertaining to its pathogenesis and therapy. It is hoped that, through this report, nevoid hyperkeratosis of the nipple and/or areola becomes a more recognizable and reported entity, thereby stimulating future studies regarding its pathogenesis and therapy.