Introduction. Skin lesions in close proximity to the lumbosacral region should be assessed in newborns, since they may be the first of hidden spinal dysraphism. Clinical case. We present the case of a newborn without significant prenatal history. On the first day of life, a 1 cm diameter nodular lesion was found at the lumbar level of the right paravertebral region, with a vascular stain surrounding the base of the lesion. Neurological examination was normal. A soft tissue ultrasonography was carried out. It showed no continuity with the lumbar spinal canal. As a result of the lesion increasingly growing, resection was decided upon six months later. Pathological examination confirmed diagnosis -Hoffmann-Zurhelle nevus.Discussion. Hoffmann-Zurhelle nevus is an infrequent cutaneous hamartomatous lesion in newborns. Treatment is always surgical in order to avoid potential growth-related complications.