A 28-week estimated gestational age infant presented to our neonatal intensive care unit at 6 days of life with intestinal perforation. Urgent surgical exploration revealed a perforated Meckel's diverticulum (MD) with no other intestinal abnormalities. Pathologic examination of the specimen demonstrated a perforated MD with no heterotopic mucosa present. According to our literature review, this is the first reported case of a perforated MD in a micropremature infant. In the English literature, there have been a total of seven reported cases of perforated MD in the last 25 years. This report summarizes those cases and characterizes the presentation, prognosis and management of this very rare complication in neonates.