Background Pulmonary arteriovenous malformations (PAVMs) are direct communications between the smaller pulmonary arteries and veins. These lesions allows blood to bypass the capillary system, flowing from arteries directly into veins. Case history The patient was a 51-year-old male, non-hypertensive, non-diabetic, asthmatic who came in due to recurrent episodes of haemoptysis. Initially this was treated as a case of pneumonia and/or pulmonary tuberculosis (PTB). A CT scan with contrast and CT angiogram were done and revealed AV malformation. He was referred to surgical service for elective thoracotomy and right middle lobe lobectomy. Histopathological findings showed lung tissue exhibiting areas of variably sized thin and partially thick vascular channels. The area was in close proximity to a bronchus and accompanying medium-sized arteries, predominantly extended to the pleural lining. Discussion The diagnosis of PAVM should be suspected in patients with any of the following presentations: (1) one or more pulmonary nodules associated with typical roentgenographic findings, (2) mucocutaneous telangiectases, and (3) unexplained findings such as dyspnoea, haemoptysis, hypoxaemia, polycythaemia, clubbing, cyanosis, cerebral embolism, or brain abscess. A practical approach would be to screen patients initially with chest radiograph, followed by shunt measurement by 100% oxygen. Our case was confirmed with PAVM by CT scan and CT angiogram and underwent right middle lobectomy. Other treatment options are embolisation and lungtransplantation. Conclusion The rarity of PAVMs and the variability of presentation posed a great challenge. Our case, in particular presenting as a non-resolving pneumonia with an unusual location, is a significant contribution to our registry. Its rarity should alert clinicians to include PAVMs in the differential diagnosis of patients with pneumonia and haemoptysis.