A 67-year-old male was admitted with cyanosis, digital clubbing and exertional dyspnea. Laboratory data revealed severe polycythaemia with 26 mg/dl hemoglobin, red blood cell; 866 x 10(-4)/mm3, hematocrit 72.8% and PaO2 44.6 mmHg. Selective pulmonary angiography demonstrated a large arteriovenous fistula involving the right middle lobe. After venesection of 1,200 ml of blood, the middle lobectomy was performed safely. In a case of pulmonary arteriovenous fistula with such severe polycythaemia, preoperative venesection is useful to decrease perioperative complications.