The objective of this study was to review all cases in literature in which the clinical manifestations of ischemic stroke and immune thrombocytopenia (ITP) were presented in the same patient including a new case of our own and discuss the possible mechanism and management of this syndrome. We reviewed 12 reports in which 18 cases were diagnosed as ischemic stroke and ITP. The clinical manifestations and ischemic lesion patterns of the 18 cases and our new case were analyzed in detail to elucidate the characteristics and management of this kind of syndrome. Of all the cases, 8 females and 10 males, 10 of them were Koreans; 3 were Americans; 3 were Japanese; 1 was British and 1 was Australian. The age of eight patients was no more than 50 years old. Most of them had a low platelet count. CT and/or MRI of brain were seen in all tested cases. Prognosis of ischemic stroke was good in 18 of the 19 patients. Although extremely rare, ischemic stroke and ITP may present in the same patient with variant characteristics. This paradoxical mechanism and management of ischemic stroke associated with ITP requires further investigation.