Nevoid hyperkeratosis of the nipple and areola is an extremely rare and benign skin disease of unknown etiology, predominantly seen in childbearing aging women, especially in the second and third decades of life. The disease is characterized by gradually growing verrucous thickening and brown pigmentation of the nipple. Here, we present a case of nevoid hyperkeratosis of the nipple and areola in a 42-year-old female patient. Although nevoid hyperkeratosis of the nipple and areola has a benign character, it is a problem for patients due to deformity and for clinicians because of its similarity to paget disease. There is no consensus on treatment; Topical agents such as keratolytics, steroids, retinoids or calcipotriol, and ablative methods such as cryotherapy, carbon dioxide laser, radiofrequency, or shaving excision are treatment options.