Intussusception is a known complication of Meckel’s diverticulum. However, intrauterine intussusception secondary to Meckel’s diverticulum has unknown incidence. Our case describes the medical and surgical management of a newborn with intrauterine intussusception of a Meckel’s diverticulum as a cause of the vascular malformation leading to ileal atresia. To our knowledge, this is the fifth case report of this phenomenon. In all cases, definitive management required exploratory laparotomy, bowel resection, and intestinal primary anastomosis.