Syringocystadenoma papilliferum is a rare benign adnexal tumor that originates from the apocrine and eccrine glands. It mainly manifests as a solitary lesion in the head and neck; however, rarely, it may involve the trunk and limbs, typically with a linear pattern. Here, we report an extremely rare case of congenital linear syringocystadenoma papilliferum on the left buttock near the anus in a 6-year-old girl. This lesion should be considered in the list of differential diagnosis of linear lesions in order to prevent complications with proper diagnosis, treatment or follow-up.
Keywords: Adnexal tumor; Apocrine gland; Eccrine gland; Linear; Syringocystadenoma papilliferum.