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Cor triatriatum is a rare congenital cardiac anomaly defined by an abnormal septation within the atrium (left or right) leading to inflow obstruction to the respective ventricles. It exists either in isolated classical form or may be associated with simple to complex congenital cardiac anomalies. Several anatomical variants exist even in the classical form, and therefore, it may require multimodal diagnostic modalities to characterize and differentiate for better percutaneous interventional or surgical planning. It commonly presents in infancy but may remain undetected till death. Symptomatology typically mimics mitral and tricuspid stenosis in sinister and dexter varieties, respectively. However, features of systemic embolization, heart failure, atrial fibrillation, cyanosis, cardiac asthma, syncope, and sudden cardiac arrest have also been reported in the literature. Surgical correction under cardiopulmonary bypass is the preferred treatment. Nevertheless, balloon dilatation may be considered in anatomically compatible variants and in special circumstances, such as heart failure, pregnancy, or as a bridge to definitive treatment.