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Appendicitis is a very rare clinical condition in neonates. Preterm and male neonates are commonly affected. It is often seen in association with surgical conditions like Hirschprung and necrotizing enterocolitis. Non-specific clinical features result in delay in the diagnosis and predisposes to perforation. The risk of perforation is very high (85%) in neonatal appendicitis. Management includes pre-operative stabilization, appendectomy and peritoneal drainage in cases of perforation with peritonitis. Prognosis is uniformly poor with mortality rate is as high as 70%. Herewith reporting a 13-day-old male twin II with a tender mass in left inguinal canal and perforated appendicitis who was successfully managed.