Introduction: Pulmonary arteriovenous malformation (PAVM) is a rare cardiovascular anomaly and represents direct communications between the branches of pulmonary artery and pulmonary veins, without an intervening pulmonary bed.The incidence of PAVM is 2-3 per 100,000 population. The male to female ratio varies from 1:1.5 to 1.8. A clinical manifestation may be from asymptomatic to severe hypoxia.Case presentation: A ten years old boy was admitted to a regional hospital because the mother noticed that the boy was slower in the game at the teacher noticed that the boy was slower and could not lift his eyes up of clinical and laboratory examinations have been shown polycythemia, and cyanosis, bat-like appearance of the fingers and polycythemia, RTG chest and CT have claimed AV malformation of the lower left lobes with the feeding artery Neurologic symptoms may be the presenting symptoms in up to 40% of patients.The patient was successfully treated with left lower lobectomy. Early post-operative recovery has been successful. Later checkups showed better blood saturation with oxygen, the same polycythemia, and a better clinical condition. Discussion and Conclusion:If the etiology of the hypoxemia often remains unclear pulmonary AVM should be considered. The diagnosis needs to be confirmed with a CT scan that should identify the food artery.If the AV malformation occupies large part of the lobe, a reasonable treatment options should be lobectomy. Long-term follow up, including chest CT examinations every 1 to 2 years, is recommended.