A 7-year-old girl with Noonan syndrome (NS) presented with a history of recurrent haemoptysis. She was diagnosed with pulmonary stenosis at the Paediatric Cardiology Department at Chris Hani Baragwanath Academic Hospital (CHBAH) and was treated with a transannular patch at the age of 6. She had severe pulmonary regurgitation following the surgery and was on treatment with furosemide, potassium chloride and digoxin. 
 She presented to her local hospital with a history of coughing up fresh red blood a year after undergoing surgery. The parents could not quantify the amount of blood; however, it was thought to be significant because she had a haemoglobin level of 7.5 g/dL and required a transfusion of packed red blood cells. It was reported that she had two other episodes of haemoptysis 11 months prior but did not seek medical attention. She was airlifted to CHBAH for further management. On arrival, she was noted to be in respiratory distress and was put on 60% O2 via a rebreathing mask. She was pink and well perfused. She had an early diastolic murmur of pulmonary regurgitation. 
 Detailed echocardiography excluded pulmonary hypertension and pulmonary vein stenosis. Laboratory investigations showed haemoglobin (11 g/dL), platelets (235 x 109 cells/L), international normalised ratio (1.02) and prothrombin time (34). She was HIV-negative and had normal urea and creatinine levels. She had a von Willebrand factor antigen of 34% (normal range 50 - 160%) and activity of 88% (normal range 66 - 99%) and her Factor VIII level was 52 IU/dL. Her sputum showed no acid-fast bacilli, a Mycobacterium tuberculosis culture was negative and no other pathogenic bacteria were isolated. A computed tomography (CT) scan of the chest showed a right lower-lobe dense consolidation, which was thought to be due to lobar pneumonia (Fig. 1). She had a rigid bronchoscopy, which also showed an inflammatory polyp at the entrance to the posterior basal segment of the right lower-lobe bronchus (Fig. 2). The inflammatory polyp and lobar pneumonia were thought to be the cause of the haemoptysis, which was exacerbated by her underlying haematological abnormalities. She was treated with antibiotics and discharged home. A repeat bronchoscopy 3 months later showed that the polyp had significantly decreased in size. 
 She presented to the local hospital 2 weeks after the repeat bronchoscopy with another episode of massive haemoptysis, where she was resuscitated with fresh frozen plasma and a platelet transfusion. She was airlifted to CHBAH after stabilisation. A repeat bronchoscopy was done, but the patient had massive haemoptysis during the procedure and required resuscitation and intensive care admission for mechanical ventilation. She was referred to the cardiothoracic team for lobectomy to control the haemoptysis. She continued to have more episodes of haemoptysis while awaiting surgery. Rigid bronchoscopy was done and did not reveal a polyp. She had a cardiac catheterisation for further investigation of the haemoptysis and an angiogram showed a torturous right bronchial artery, with extravasation of blood into the right lower lobe. The patient was referred for embolisation of the torturous bronchial artery. A descending aorta angiogram in the anterior-posterior view confirmed the findings (Fig. 3). The prominent bronchial artery formed a confluence with an abnormal vessel that arises from the right common carotid artery 
 She had a right bronchial artery embolisation and was stable post procedure. That evening she bled again during rigid bronchoscopy. The decision was taken to do a lobectomy to control the bleeding. The histology of the resected lobe showed normal lung parenchyma, with patchy bronchopneumonia, alveolar haemorrhage and fibrointimal hyperplasia of the hilar vessels. The bronchus at the resection margin was normal and patent. There was no granulomatous inflammation or malignant neoplasm. The patient had an uneventful course post lobectomy, and after 6 months follow-up there was no further bleeding reported.