In this report, we descrbed a case of multiple brain abscesses associated with diffuse congenital pulmonary arteriovenous malformations (PAVM). Although the cases of brain abscesses associated with congenital PAVM are very rare, the brain abscess could be an initial clinical manifestation in asymptomatic PAVM as in the case presented in this report. PAVM may contribute to the development of a brain abscess by allowing easy bacterial access to systemic circulation through the right-to-left pulmonary vascular shunt, bypassing the filtering effect of the pulmonary capillaries. Hence, this association should be considered in cases with brain abscesses of undetermined etiologic factors.