Myelodysplastic syndrome (MDS) is a bone marrow failure syndrome characterized by cytopenia that results in infection and bleeding. However, there are few reports of cerebral infarction in MDS. In this case report we reported a Chinese female patient diagnosed MDS without drugs and an onset of acute cerebral infarction. Imaging examinations showed an ischemic stroke and further bone marrow aspiration identified MDS in the patient. Low dose aspirin and rehydration were used to improve symptom, as well as anti-epileptic drugs and rehabilitation. We also reviewed acute cerebral infarction associated with MDS from a total of three reported cases without drugs for the treatment of MDS. Our data provide further evidence that acute ischemic stroke might be associated with MDS, which may be due to complex chromosomal abnormality and inflammatory processes.