Pulmonary arteriovenous malformations (PAVMs) are abnormal connections between the pulmonary artery and pulmonary vein bypassing the normal capillary bed causing a right-to-left shunt. The majority (80–90%) of PAVMs are associated with hereditary hemorrhagic telangiectasia (HHT). PAVMs may be asymptomatic or present with symptoms of hypoxia, shortness of breath, migraines, sequelae of paradoxical embolization, or rupture. Transcatheter embolization has become the standard of care. This article will review the clinical presentation, workup, genetics, imaging findings, embolization, complications, and follow-up for patients with PAVMs.