Nevus sebaceous is a benign, congenital hamartoma of the skin, with a predilection for the scalp and less commonly occurs on the face, around the ears, neck or on the trunk. In late childhood and early adulthood, there is a significant risk of developing secondary tumors, the most common of which are__ampersandsignnbsp;__ampersandsignnbsp;syringocystadenoma papilliferum and basal cell carcinoma. A case of syringocystadenoma papilliferum associated with nevus sebaceous in a 26 year old female patient in the nipple areolar region. A rare site of occurance of syringocystadenoma papilliferum arising in nevus sebaceous is described. To the best of our knowledge, this appears to be the first case reported in the nipple areolar complex.