Introduction: Arteriovenous malformations (AVM) are rare chronic congenital diseases whose nature are not yet fully understood. Two different types of these disorders are pulmonary and brain malformations, which are seen sporadically or in combination with other syndromes. Symptoms of these diseases appear at different ages and present differently in patients, but in general, the combination of these two disorders is rarely seen. There are several treatments based on the type of lesion and symptoms and other accompanying conditions, one of which is interventional procedure. Case Presentation: In this article, a rare case of pulmonary and brain malformation has been reported, which has progressed even after treatment and has been closed interventionally. Conclusions: Patients with pulmonary AVMs need careful examination for other congenital disorders. Furthermore, due to the unknown nature of the disease and its progression, the treatment of these lesions is completely personal and varies according to each patient.