Acute appendicitis is a rare condition in neonates, with a high mortality. If perforated, it seldom presents with radiologically significant pneumoperitoneum. An 11-day-old newborn presented with abdominal distension and reluctance to feeds. X-ray abdomen revealed significant pneumoperitoneum. After optimization of his condition, exploratory laparotomy was performed. Perforated appendix was found and appendicectomy done. Post operative course was stormy that lead to demise of the baby.