Background Nutcracker phenomenon (NCP) can occur due to various anatomical anomalies. Anterior and posterior NCP are defined in the literature. Posterior NCP combined with left atrial isomerism is a rare condition. Case description We present a rare case of an asymptomatic posterior NCP involving the azygos vein in a patient with a complex cardiovascular pathology with left atrial isomerism, left ventricular outflow tract obstruction, interrupted inferior vena cava, and azygos continuation. Conclusion Detection of the NCP especially involving such a rare anatomical anomaly as an azygos continuation has a crucial importance for diagnostic and surgical procedures.