Venous drainage to the portal vein in pulmonary sequestration is rare. A 7-month-old girl was referred to our hospital following surgery for ventricular septal defect because of a left upper abdominal mass with a large feeding artery from the abdominal aorta and venous drainage to the portal vein. She had had frequent pulmonary infections and was growth retarded. MRI demonstrated that the mass was above the left diaphragm, suggesting extralobar sequestration. An extralobar sequestered lung was resected at thoracotomy. Diagnostic problems and clinical features are presented.