Perforation is a rare complication of Meckel`s diverticulum (MD), but it could be severe, even life-threatening for pediatric patients. There is a paucity of data on etiology of perforation, as well as clinical manifestations, management and prognosis in very low birth weight (VLBW) neonates with perforated MD. We report a rare case of spontaneously perforated MD in a VLBW neonate presenting with severe pneumoperitoneum. To our knowledge, this is one of the earliest reported VLBW cases with this rare complication. Furthermore, we review relevant publications and summarize major characteristics of all VLBW cases previously reported in order to provide some practical experience and interesting issues for pediatricians. Perforated MD should be kept in mind when VLBW neonates present with pneumoperitoneum.

Keywords: Meckel`s diverticulum; intestinal perforation; very low birth weight; neonates; pneumoperitoneum