An infant, nine days old, with total anomalous pulmonary venous drainage into the portal vein was examined. The exact anatomy was clarified by means of cardiac catheterization and angiocardiography. One day later the operation was performed utilizing circulatory arrest in deep hypothermia. The procedure was successful, and the child, two years old now, is doing well. The problems of examination, treatment and surgery are discussed. There are only a few cases with successful correction in the literature.