Pulmonary arteriovenous fistula (PAVF) is a rare congenital vascular malformation primarily manifested as dyspnea, migraine, ischemic stroke, hemoptysis, and nervous system complications. However, in our case, an 18-year-old male patient with PAVF presented with sudden onset of ventricular tachycardia and type 2 acute myocardial infarction as initial symptoms. A diagnosis was achieved through pulmonary artery computer tomography angiography (CTA) and three-dimensional (3D) computed tomography (CT) reconstruction, revealing a complex and giant PAVF. Following multidisciplinary team (MDT) consultation, the patient underwent thoracoscopic surgery and experienced a successful recovery during follow-up.