ST-elevation myocardial infarction (STEMI) as a complication of infective endocarditis (IE) is a rarely reported entity. No clear guidelines exist with regards to the management of this medical emergency. We sought to systematically review the clinical presentation and management of this condition. We searched relevant articles on STEMI associated with IE and extracted data on demographic variables, key clinical characteristics upon presentation, treatment strategies, and clinical outcomes. We identified 100 patients from 95 articles. The mean age at presentation was 53 ± 17 years with male preponderance (n = 63, 63%, p = 0.01). Most patients (63 of 100, 63%) presented with STEMI as their first manifestation of IE, with others occurring at 15 ± 17 days after diagnosis of IE. Findings that suggested possible septic emboli were not consistently present, including history of prosthetic valve placement (15%), presence of other embolic disease (27%), fever (42%) increased leukocyte count (80%), and presence of murmur (88%). Atherosclerotic disease was absent in 95% on cardiac catheterization. Eleven patients receiving tissue plasminogen activator fared poorly, with 9 major bleeds; balloon angioplasty was successful in 56% (9 of 16 cases), aspiration thombectomy in 68% (21 of 31 cases), and coronary stenting in 81% (14 of 16 cases). The 30-day mortality was 43%. In conclusion, patients with STEMI in the face of recent IE, new precordial murmur, fever, increased leukocyte count or other embolic events, septic emboli should be considered as a cause for STEMI. Best practices for management are not known, but thrombolytics appear to carry significant bleeding and embolic risks.