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A retrospective analysis was conducted on the baseline characteristics, clinical symptoms and signs, laboratory and imaging findings, and treatment prognosis of 10 PAVF patients from Northern Jiangsu People's Hospital from February 2013 to November 2024. The age of onset was between 16 and 79 years, with 3 cases in men and 7 cases in women. Among the 10 patients, 9 had pulmonary arteriovenous fistula and 1 bronchial artery-pulmonary vein fistula. Clinically, there were 4 cases of dyspnea and chest tightness, 3 cases of cough and sputum production, 1 case of chest pain, 2 cases of hemoptysis, 1 case of dizziness, 2 cases of nosebleeds, 1 case of multiple red nevi on the skin, 3 cases of asymptomatic findings upon physical examination, and 3 cases of acute cerebral infarction as the onset. Patients are mainly diagnosed with PAVF based on chest contrast-enhanced CT and/or pulmonary artery CT angiography (CTA). In terms of treatment, 6 cases underwent interventional embolization; 2 patients underwent surgical treatment: one underwent thoracotomy to remove the diseased lob, and one underwent video-assisted thoracoscopic lobectomy; 2 patients received conservative treatment. 10 patients improved and were discharged after treatment, with follow-up lasting 2.1-4.5 years. Among them, one complex PAVF patient was admitted one year after surgery for a second interventional treatment. During follow-up, 10 patients had stable conditions with no related complications, and no recurrence or signs of vascular embolization or recanalization were observed. Clinical manifestations of PAVF patients are diverse, diagnosis requires chest contrast-enhanced CT and pulmonary artery CTA, treatment methods include interventional embolization therapy and surgical treatment, follow-up is required after surgery to evaluate treatment effectiveness.