Intrauterine appendicular perforation leading to meconium peritonitis is exceptionally rare, with few reported cases in the literature. This case underscores the diagnostic challenges and high mortality associated with neonatal appendicular perforation. Neonatal appendicitis and subsequent perforation are uncommon due to the funnel shape of the fetal appendix, which reduces susceptibility to luminal obstruction. While advances in neonatal care and diagnostic modalities have improved outcomes, challenges persist in timely diagnosis and management. We present the case of a preterm infant, one of dichorionic-diamniotic (DCDA) twins delivered via cesarean section, who developed gross abdominal distension and respiratory distress shortly after birth. Diagnostic abdominocentesis revealed meconium-stained fluid, prompting further investigation with imaging and subsequent exploratory laparotomy. Extensive adhesions and cecal perforation were observed, necessitating a cecostomy. Despite interventions, the infant's condition deteriorated, leading to a fatal outcome. Intrauterine appendicular perforation leading to meconium peritonitis is a rare and difficult-to-diagnose condition. Antenatal suspicion and early surgical intervention are crucial for improving outcomes. Factors contributing to neonatal appendicular perforation include ischemia, obstruction, and infective etiologies. Neonatal appendicular perforation is a rare but life-threatening condition requiring a high index of suspicion for prompt diagnosis and management. Advances in diagnostic tools and antenatal monitoring have contributed to improved outcomes, highlighting the importance of considering this diagnosis in cases of unexplained neonatal abdominal distension.