Background: Heterotaxy is a condition of abnormal lateralization of organs across the body's left-right axis, causing multiple congenital malformations. The anatomic manifestations of heterotaxy syndrome generally follow one of two patterns, referred to as right atrial isomerism (with two similar right atria and duplication of right-sided features of multiple organs) and left atrial isomerism (with two similar left atria and duplication of left-sided features of multiple organs). Cardiac surgical intervention for patients with heterotaxy syndrome depends on ventricular physiology and circulatory balance. For patients with single-ventricle physiology, a Fontan operation, which directs systemic venous return to the pulmonary arteries, is the definitive intervention. Prior to a Fontan operation, many patients require one or more palliative surgeries (eg, a Blalock-Taussig-Thomas shunt or bidirectional Glenn/Kawashima procedure) to prepare them for definitive correction.; Case Report: We present the case of a term female neonate who was transferred to our pediatric cardiovascular intensive care unit for management of suspected congenital cardiac disease. Echocardiography confirmed the diagnosis of heterotaxy syndrome with left atrial isomerism, an interrupted inferior vena cava with azygos continuation, and a hypoplastic left ventricle with single-ventricle physiology. At 11 months of age, she underwent a Kawashima procedure with subtotal pulmonary artery ligation. She tolerated the procedure well and is anticipated to remain stable for the near future, possibly without the need for further cardiac surgery.; Conclusion: Patients with heterotaxy syndrome have congenital malformations in several organ systems, requiring lifelong coordination of care among health providers across multiple disciplines.