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Cor triatriatum (CT) otherwise known as a tri atrial heart is a rare congenital heart defect. This report describes a rare case of CT dexter in a 2-year-old girl who presented to our facility with cough and difficulty in breathing of a year duration and body swelling of 3 months duration. She was also found to be in heart failure. Transthoracic echocardiography revealed a membrane separating the right atrium into two chambers, with features of pulmonary hypertension. Although the patient was scheduled for follow-up, she however, died 2 days later. Early diagnosis and prompt referral of patients with this rare defect are mandatory to prevent mortality from this defect that is amenable to simple corrective surgery.