A35-year-old woman with a medical history of hereditary and recurrent epistaxis and lip telangiectasia was admitted with sudden left thoracic pain and dyspnea. There was no recent history of fever or thoracic trauma. Biologic data were as follows: hemoglobin level, 8.8 g/L; hematocrit level, 28%; oxygen saturation, 97%; and fraction of inspired oxygen, 10 L/min. Chest radiography and computed tomographic (CT) scanning revealed a left hemothorax with a suspicion of a vascular malformation in the left lower lobe (Figure 1). There were no aortic or pericardial abnormalities. An intercostal drain was placed in the left pleural cavity, and 2000 mL of blood was drained, leading to a significant clinical improvement. Pulmonary angiography confirmed the presence of 2 arteriovenous fistulas in the upper part of the left lower lung and in the lingula (Figure 2). Rendu-Osler-Weber syndrome (ROW) was diagnosed on the basis of the patient’s history and clinical and radiologic data. Embolization of the lower lobe fistula was performed during general anesthesia with 3 Guglielmi Detachable Coils (Boston Scientific, Target Therapeutics, Fremont, Calif). One 3-dimensional shaped coil of 8 20 mm and 2 fibered coils of 6 20 mm and 4 20 mm, respectively, were placed with a 0.018-inch microcatheter (Boston Scientific, Target Therapeutics) in the feeding artery. Occlusion was completed with the injection of 0.3 mL of enbucrilate (Histoacryl; Braun Aesculap, Tuttlingen, Germany) emulsified with iodized oil (Lipiodol; Guerbet, Aulnay-sous-Bois, France). Catheterization of the lingula fistula was technically more difficult, and occlusion was achieved with a 4 20–mm fibered spiral placed with a 0.018-inch microcatheter (Boston Scientific; Figure 3). Immediately after the endovascular procedure, video-assisted thoracoscopic surgery was performed to remove any residual clot and exclude further bleeding. The patient was placed in the right decubitus position. A single-lumen endotracheal tube was inserted, and ventilation was performed with reduced tidal volume throughout the procedure. A 10-mm trocar was introduced through a skin incision into the fifth intercostal space in the midaxillary line for insertion of a 0° endoscope (Karl Storz, Tuttlingen, Germany). Numerous clots and a large hemothorax in the left thoracic cavity were observed. Two additional ports were then inserted under direct vision. After the entire thoracic cavity had been rinsed with saline and clots had been removed, pulmonary arteriovenous fistulas were visible on the surface of the lung (Figure 4). No active bleeding was observed, and no further operations were required. Two 28F chest drains were introduced through the trocar incisions, one in the eighth and one in the anterior fifth intercostal spaces. The tubes were connected to an underwater seal suction device (Pleurevac; Genzime, Cergy-Pontoise, France) with a negative pressure of 20 cm H2O. The patient was extubated 3 hours after the operation, and pain was managed with a patient-controlled analgesia pump. Intercostal drainage was continued for 4 days, with a total volume of 975 mL. No air leak was observed, and the postoperative hospital stay was 6 days. Follow-up angiography at 1 month and CT scanning at 3 months revealed recurrent limited flow in the lingula fistula (Figure 5). Repeat embolization was performed with 2 coils (1 fibered coil of 2 50 mm and 1 coil of 2 30 mm) placed with a 0.018-inch microcatheter (Boston Scientific). The occlusion was completed without injection of enbucrilate. Follow-up CT scans at 3 months and 2 years showed no recurrence.