Background; Congenital diaphragmatic hernia is a life-threatening condition caused by a defect in the diaphragm, allowing abdominal organs to herniate into the thoracic cavity, leading to pulmonary hypoplasia and respiratory distress. The association of congenital diaphragmatic hernia with pectus excavatum and dextrocardia without situs inversus is exceptionally rare. Dextrocardia in this context results from a primary cardiac malformation. Early recognition and prompt surgical intervention are crucial to prevent severe complications and improve survival outcomes. Case Presentation; We report a six-week-old infant presenting with features of cardiopulmonary distress. The baby exhibited tachypnea (respiratory rate: 75 breaths/min), tachycardia (pulse rate: 185 beats/min), and mild desaturation (96% on oxygen therapy via nasal prongs). On physical examination revealed a normal abdominal contour with normal bowel sounds. Chest Computed Tomography scan and echocardiography confirmed left-sided congenital diaphragmatic hernia with pectus excavatum, and dextrocardia without situs inversus. The patient underwent an emergency laparotomy, where intraoperative findings included a significant diaphragmatic defect with herniated abdominal viscera within the thoracic cavity. Surgical repair involved reduction of herniated contents and primary diaphragmatic closure. Postoperatively, the infant received ventilatory support, intravenous fluids, and antibiotics. The recovery was uneventful, with gradual weaning off respiratory support and subsequent discharge in stable condition. Discussion; The incidence of CDH is approximately 1 in 4,000 births, with Bochdalek hernia being the most common type (85%). Delayed surgical intervention increases the risk of life-threatening complications. Conclusion; This rare case highlights the importance of early diagnosis, multidisciplinary management, and timely surgical intervention for optimal outcomes.
