The association of acute myeloid leukemia (AML) and acute myocardial infarction (AMI) is rare. We encountered a 40-year-old female with inferior wall myocardial infarction that occurred simultaneously with the diagnosis of AML. She developed subsequent dural sinus thrombosis during chemotherapy for AML. The screen for thrombophilia revealed that she had hyperhomocysteinemia. In the English literature, only 4 cases have been reported previously. Remission induction was not affected by the occurrence of AMI, although anthracyclines were avoided in all cases. In the absence of conventional risk factors for coronary artery disease, AMI can be related to leukemia per se and the role of homocysteine is worth further investigation.