Zosteriform connective tissue nevus is a rare form of connective tissue hamartomas, which arises from cells of mesodermal origin. Despite similar clinical appearance of many connective tissue nevi, they can be differentiated histochemically and/or biochemically on the basis of the primary connective tissue element present. There are only 3 reported cases of zosteriform connective tissue nevi in the worldwide literature. We report a case occurring in a 25-year-old male with approximately 40 nodules and smaller papules distributed in a zosteriform fashion on the right lower lumbar region and upper gluteal region. The identification of the lesion by deep biopsy excluded the important differential diagnosis of segmental neurofibromatosis.