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Neonatal appendicitis is a rare and often life-threatening condition due to its non-specific presentation and diagnostic overlap with other neonatal abdominal pathologies. We describe a case of a previously healthy full-term female neonate who presented with fever and progressive abdominal distension. Imaging revealed pneumoperitoneum, prompting emergency exploratory laparotomy, which identified a perforated appendix with no other anomalies. An appendectomy was performed, and the patient recovered uneventfully with intravenous antibiotics and supportive care. We also present a brief review of the literature to highlight the diagnostic challenges, role of imaging and the importance of early surgical intervention in improving outcomes for this rare condition.