Meckel’s diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract. It is usually encountered as an incidental finding at operation or autopsy. Symptomatic cases usually present during infancy with intestinal obstruction, intestinal hemorrhage, diverticulitis, or perforation. We report on a preterm newborn who developed abdominal distension at 17 h of age and pneumoperitoneum at 30 h. At laparotomy, a narrow-based MD with a small perforation at the tip was encountered. Segmental resection of the ileum, including the MD, and end-to-end anastomosis was performed. A review on perforated MD from the English medical literature is also presented. Perforated MD, although rare, should be included in the differential diagnosis of neonatal gastrointestinal perforations and acute abdomen.