Multiple pulmonary arteriovenous malformations (PAVM) constitute an uncommon cause of respiratory disability. They may lead to severe hypoxaemia via right-to-left shunts and are sources of substantial mortality and morbidity. Conservative surgical resection has been proposed as the treatment of choice. More recently, percutaneous balloon or coil embolization of the feeding vessels offered an efficacious and safe alternative therapy for patients whose fistulas are too numerous to excise. This study reports an unusual case of respiratory disability in a patient with multiple and microscopic pulmonary arteriovenous malformations who failed to respond to embolotherapy and who received a double lung transplantation with good initial outcome.