Nevus sebaceous is a benign hamartoma with epidermal, follicular, and apocrine elements and has a well-documented neoplastic potential. Several appendageal tumors have been described presenting within a nevus sebaceous. Syringocystadenoma papilliferum (SCAP) is a rare benign adnexal neoplasm that presented hamartomatous malformation of apocrine and eccrine sweat glands, and occur often in conjunction with a nevus sebaceous. The patient was a 31-year-old female who presented with 6.0 x 1.0 cm sized normal skin colored to yellowish linear verrucous plaques, nodule on the left forehead since childhood. Histopathological examination of a skin biopsy specimen from the lesion revealed 2 distinctly different findings. The nodular portion of lesion showed cystic invaginations extending into the dermis, with numerous papillary projections. The plaque portion of lesion showed papillomatosis, acanthosis in the epidermis, and consisted of many mature sebaceous glands, several ectopic apocrine gland in the dermis. We diagnosed this case as linear nevus sebaceous associated with SCAP on the basis of above clinical and histopathological examination. The patient was treated with surgical excision. The recurrence has not been observed during 3 months of follow up.