PAVMs or fistulae are abnormal vascular structures characterized by low resistance and high flow, which bypass the pulmonary capillary system. In this condition, the arterial system is directly connected to the venous system, resulting in the creation of an intrapulmonary right-to-left shunt. This allows systemic venous blood to circumvent gas exchange and processing by the pulmonary capillary bed, leading to hypoxemia. HHT is the primary underlying cause. Given the high risk of systemic thromboembolism and hypoxemia in patients with significant shunts, all treatment options—whether transcatheter or surgical—should be considered. Currently, transcatheter feeding artery occlusion (for arteries with a diameter > 3 mm) is regarded as the gold standard for treatment. Due to the considerable risk of recanalization, the occlusive device should be placed as distally as possible. In many instances, multiple fistulae are identified, necessitating a multi-staged approach. Meticulous follow-up is essential for these patients, particularly during pregnancy.