A case of subtotal cor triatriatum associated with a partial anomalous pulmonary venous drainage of the left lung, in a 30 months old boy is reported. Diagnosis was suggested by the presence of pulmonary venous obstruction and pulmonary hypertension. Doppler, echocardiogram diagnosed the cor triatriatum and the degree of the pulmonary hypertension. The cine-angiography demonstrated the anomalous, obstructive, left pulmonary venous drainage. The pulmonary hypertension has mandatorised an early surgical cure, the result was satisfactory at 16 months.