A 25-year-old male patient presented with thickened rough surfaced black pigmentation around both areolae which had been present for 10 years. He had no underlying disease and medication history. Histopathological findings showed hyperkeratosis, papillomatosis, acanthosis, keratotic plugging, and mild perivascular lymphocytic infiltrate in the dermis, consistent with nevoid hyperkeratosis of the nipple or areolar. Nevoid hyperkeratosis of the nipple or areolar (NHNA) is a rare, idiopathic disorder. It is characterized by slowly growing verrucous thickening and brown pigmentation of the areola or nipple. Levy-Frankel described three distinct types of hyperkeratosis of the nipple and/or areola. Type I is associated with an epidermal nevus. Type II is hyperkeratosis associated with other dermatoses, such as acanthosis nigricans, ichthyosis. Type III is nevoid hyperkeratosis and has no association with other dermatoses. The third type, called nevoid hyperkeratosis, is usually bilateral, asymptomatic and appears either at puberty or during pregnancy in females. Although it affects both men and women, very few men have been reported to have nevoid hyperkeratosis, and most had a medical history of prostate adenocarcinoma treated by estrogen therapy. Herein we report a case of nevoid hyperkeratosis of nipple and areola in a male patient.