We describe a case of a 38-year-old female with pulmonary arteriovenous fistula (PAVF), who underwent successful transcatheter occlusion of the effluent vein via a transpulmonary venous approach with transseptal puncture. The patient presented with cough and shortness of breath from exercise. Cyanosis and clubbed finger were observed. A continuous murmur was heard at the third to fourth intercostal space over the left sternal border. Computer tomography angiography detected a single PAVF with 2 tortuous feeding arteries and 1 straight effluent vein in the left upper lobe. The delivery sheath and guide wire failed to be advanced into the fistula via transpulmonary arterial approach due to the tortuous feeding arteries. The PAVF had a wide and straight effluent vein that drained the fistula. Transseptal puncture in the atrial septum was performed with a transseptal sheath. The PDA occluder was advanced through the stretched flap into the effluent vein via a transpulmonary venous approach. Occluder deployment to occlude the effluent vein increased the arterial partial pressure of oxygen and preceded the disappearance of murmur. We conclude that transcatheter occlusion of the effluent vein is effective for closure of the PAVF with transseptal puncture. The transpulmonary venous approach is a feasible alternative, in cases with tortuous feeding arteries and a straight, dilated effluent vein that only drains the fistula.