Introduction: Pentalogy of Cantrell is a rare, congenital disorder characterized by lower sternal defects, diaphragmatic defect, pericardial defect, supraumbilical abdominal wall abnormalities, and/or intracardiac defects. The collective defects result from the failure of either differentiation or migration of mesenchymal or mesodermal structures during the embryonic phase of development. Mortality of the disease complex is high, and treatment, when appropriate, revolves around the surgical correction of the associated defects. Objective: This article presents a case of pentalogy of Cantrell and examines the literature to report the most current evidence relative to embryology and pathophysiology. Methods: Case report was gathered from the OPD medical records and is provided as it occurred. The literature was searched for evidence of best management strategies as well as care implications for families. Results: A newborn was delivered at 20 weeks__ampersandsignrsquo; gestation secondary to termination of pregnancy. Prenatal ultrasonography identified an abdominal wall defect, diaphragmatic hernia, sternal defect, ventricular septal defect, kyphoscoliosis and club foot. Examination immediately after delivery confirmed prenatal findings and a diagnosis of pentalogy of Cantrell was assigned. Conclusion: Pentalogy of Cantrell is a syndrome inclusive of five anomalies: a midline, upper abdominal wall disorder; lower sternal defect; anterior diaphragmatic defect; diaphragmatic pericardial defect, and congenital abnormalities of the heart. The pathogenesis of this condition is unclear, with associations being common and extremely challenging treatment. Prognosis and outcome of the condition depend on the type of pentalogy of Cantrell and the cardiac anomalies associated with it. Diagnosis can be made reliably by antenatal ultrasound during the first trimester. In this case report, we describe a fetus of undetermined sex who had the typical features of the very rare pentalogy of Cantrell. Patients with the diagnosis of pentalogy of Cantrell should receive antenatal counselling relative to mortality and morbidity risks. An interprofessional approach in the immediate timeframe after delivery facilitates timely diagnostics and offers families prompt confirmation of antenatal findings.