Nevus sebaceous is a common congenital hamartoma developing mainly on the scalp and face, as a linear arrangement of yellowish, waxy and verrucous plaque. It has a potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. Syringocystadenoma papilliferum is most common benign tumor associated with these neoplasms. It is a rare benign adnexal tumor occurs in childhood or adolescence, typically found on the scalp, neck and face. It presents as a solitary papule, several papules in a linear arrangement, or as a plaque that may be exudative. It has a tendency to increase in size during adolescence, becoming more verrucous and papillomatous. Histologically, it consists of a cystic and papillary growth of epithelial elements projecting downward into the dermis and opening onto the skin surface through one or more orifices. A 17-year-old woman presented with grouped crusted yellowish papules in a linear arrangement on the left cheek since birth. Histopathologic findings showed endophytic papillary invaginations of the epithelium into the dermis, forming cystic spaces with hyperplastic and hypertrophic sebaceous glands. The patient was diagnosed with syringocystadenoma papilliferum arising in nevus sebaceous and observed without any treatment. Herein we report a case of syringocystadenoma papilliferum arising in nevus sebaceous.