Abstract： Objective To report 4 cases of nevoid hyperkeratosis of the nipple and areola.Methods Clinical and histopathological manifestations of 4 cases of nevoid hyperkeratosis of the nipple and areola were observed and retrospectively analyzed.Results Of the 4 patients,1 was male and 3 were female.The average age and disease duration were 32 and 4.5 years respectively.All cases showed hyperkeratosis and verrucous hyperplasia of the nipple and/or areola.Histopathology showed epidermal hyperkeratosis,papillomatous hyperplasia and elongation of the epidermal rete ridges.Conclusions Nevoid hyperkeratosis of the nipple and areola,a subtype of the hyperkeratosis of the nipple and areola,is uncommon in clinic and occurs mainly in young women with characteristic clinical and histological features.