The scintigraphic findings in a patient with multiple pulmonary arteriovenous malformations (AVMs) complicated by superimposed pulmonary embolism are reported. Although pulmonary AVMs may cause small subsegmental scintigraphic abnormalities, the demonstration of perfusion defects which are segmental or larger should strongly suggest the presence of superimposed pulmonary embolism in a patient with multiple pulmonary AVMs.