Pulmonary arteriovenous malformations (AVMs) with systemic arterial collateralization related to a prior tuberculosis empyema are extremely rare. We report the case of a 15-year-old boy who developed a pulmonary AVM with massive systemic arterial collateralization 5 years after being treated for a Mycobacterium tuberculosis empyema necessitans. The AVM was successfully managed with combined intraarterial embolization and surgical resection.