A 40-year-old man admitted to our hospital of brain abscess exhibited moderate arterial hypoxemia. Chest CT scan showed diffuse multiple arteriovenous malformations (AVMs) in the bilateral lungs and arterioportal shunts in the liver. He had also been treated for multiple epithelioid hemangiomas in the skin. There was no mutation detected in endoglin (ENG) or activin A receptor type II-like kinase 1 (ACVRL1) gene, and the diagnosis of hereditary hemorrhagic telangiectasia could not be confirmed. A coil embolization of major AVMs (6 AVMs in the right lung and 5 AVMs in the left) was performed without significant decrease in the shunt fraction. He felt numbness on his lower legs, and peripheral blood eosinophilia, elevated serum IgE levels, and positive MPO-ANCA were observed. Systemic treatment with corticosteroid improved the numbness, eosinophilia, and MPO-ANCA. This is the first report of a case with multiple vascular anomaly including pulmonary AVMs, accompanied by peripheral blood eosinophilia and ANCA-positive peripheral neuropathy.