The segmental spinal dysgenesis (SSD) is a rare and complex spinal dysraphism. We document a case of a type II SSD in a full-term female neonate with her clinical symptoms, imaging characteristics and intraoperative findings, depicting the rare association with a pure meningocele and its primary management. In addition, we performed a literature review, with special emphasis on the embryological and radiological classification, aiming to a proper diagnostic and therapeutic approach of this entity.