A pulmonary arteriovenous malformation (PAVM) is a fistulous connection between a pulmonary artery and a pulmonary vein that bypasses the normal pulmonary capillary bed resulting in a right-to-left shunt. Because of the potential for paradoxical emboli, PAVMs are treated when their feeding arteries exceed 3 mm or patients are symptomatic. PAVMs are often encountered in patients with suspected hereditary hemorrhagic telangiectasia (HHT). Sporadic cases are uncommon. The radiologist may be called on to diagnose a PAVM after positive transthoracic contrast-enhanced echocardiography in a patient with suspected HHT to direct patient management and avoid potential complications. The radiologist may also be required to evaluate a potential PAVM detected at CT performed for other reasons. Through the authors’ experiences at an HHT Center of Excellence in an area endemic with histoplas-mosis, the authors have gained a unique perspective on the diagnosis of PAVMs and differentiation of PAVMs from their mimics. Understanding the CT appearance of PAVMs limits misdiagnosis, directs appropriate treatment, and allows subsequent family screening for HHT (and avoidance of unnecessary screening when a PAVM mimic is encountered). Both vascular and nonvascular pulmonary lesions can mimic PAVMs. Vascular mimics include fibros-ing mediastinitis, venovenous collaterals, arterial collaterals, pulmonary artery pseudoaneurysms, hepatopulmonary vessels, Sheehan vessels, meandering pulmonary veins, and pulmonary vein varices. Nonvascular mimics include granulomas, nodules, mucoceles, bronchoceles, ground-glass opacities, and atelectasis. The authors review the CT technique for evaluating PAVMs and the appearance of PAVMs and their mimics.