Syringocystadenoma papilliferum is a rare cutaneous adnexal tumor that usually arises in the head and neck region. It may develop de novo or within a nevus sebaceus. Linear syringocystadenoma papilliferum is an uncommon variant of this benign tumor. We report a child with linear retroauricular distribution of syringocystadenoma papilliferum. A background nevus sebaceus was shown histologically. Total excision was curative with no recurrence. An association between the linear variant of syringocystadenoma papilliferum and nevus sebaceus has not been reported previously.