Evans syndrome (ES) is a rare hematological disease characterized by autoimmune hemolytic anemia, immune thrombocytopenia, and/or neutropenia, all of which may be seen simultaneously or subsequently. Thrombotic events in ES are uncommon. Furthermore, non-ST segment-elevation myocardial infarction (NSTEMI) during ES is a very rare condition. Here, we describe a case of a 69-year-old female patient presenting with NSTEMI and ES. Revascularization via percutaneous coronary intervention (PCI) was scheduled and performed. Hemopericardium and cardiac tamponade occurred 5 h after PCI, and urgent pericardiocentesis was performed. Follow-up was uneventful, and the patient was safely discharged. Early recognition and appropriate management of NSTEMI is crucial to prevent morbidity and mortality. Coexistence of NSTEMI and ES, which is associated with increased bleeding risk, is a challenging scenario and these patients should be closely monitored in order to achieve early recognition and treatment of complications.