Pulmonary arteriovenous malformations are rare, mostly asymptomatic vascular anomalies. These malformations cause direct right to left shunting of unoxygenated blood, resulting chronically in cyanosis, dyspnea, and exercise intolerance. The serious complications reported earlier have mainly been neurologic, for example, cerebrovascular accidents and brain abscesses and are most likely caused by paradoxical embolism. Detection of pulmonary arteriovenous malformations is challenging, and the emergency department personnel play an essential role. Once suspected, the diagnosis can be made promptly using standard imaging techniques and appropriate treatment may prevent serious complications. In this report, we describe a case of a 41-year-old man with acute anterior myocardial infarction, unexpectedly caused by emboli from pulmonary arteriovenous malformations. This rare etiology of acute myocardial infarction should be taken into consideration especially in cases with atypical presentation.