Background: As a result of its low occurrence, most of the studies on intracranial aneurysms associated with MCA anomalies were presented as case reports or small case series. In this study, a systematic review on this specific entity was conducted.Methods: A PubMed search of the published studies was performed on April 6th, 2019 for patients who had intracranial aneurysms associated with MCA anomalies. The languages included in this study were English, Chinese, and Japanese.Results: Finally, 58 articles reporting of 67 patients including 1 case in our center were included. The identified patients (37 females, 55.2%) aged from 4 to 81 (49.85 ± 15.22) years old. Fifty (50/67, 74.6%) patients presented with hemorrhagic stroke either from the MCA anomalies associated aneurysms or other sources. Sixty-three aneurysms (63/67, 94.0%) were saccular, 3 (4.5%) were dissecting or fusiform, and 1 (1.5%) was pseudoaneurysm. Thirty-two (32/65, 49.2%) patients had other concurrent cerebrovascular anomalies. Fifty-six (83.6%) patients underwent open surgeries, 8 (11.9%) patients underwent endovascular treatment, and 3 (4.5%) patients were conservatively managed. Fifty-six (56/61, 91.8%) patients achieved a good recovery.Conclusions: The pathophysiological genesis of MCA anomalies associated aneurysms is still obscure to us. The inflicted patients tend to have other concurrent cerebrovascular anomalies, which denotes that congenital defect in cerebrovascular development might play a role in this process. Open surgery is, hitherto, the mainstay of treatment for this specific entity. Most of the affected patients could experience a good recovery after treatment.