Objective: To systematically review the etiology of anterior choroidal artery (AChA) infarction. Methods: A systematic literature search up to May 11, 2024, for AChA infarction with its etiology. Epidemiologic and clinical data of patients, anatomic distribution of the lesions, and etiologic classification of AChA infarction were extracted. Results: A total of 1 007 individual patient data was included (967 from retrospective clinical studies and 40 from case reports). Among the clinical research, patients’ mean age was 64.7. There were 62.24% of male and 37.76% of female patients. Hypertension (66.04%) was the most common risk factor for patients with AChA infarction. Dyslipidemia (32.92%), diabetes mellitus (30.93%), and smoking (26.54%) were also common risk factors. Moreover, the posterior limb of the internal capsule was the most frequently affected structure. Undetermined etiology (n =173, 38.02%), according to the trial of org 10172 in acute stroke treatment (TOAST) etiological classification, was the most common etiology, followed by small vessel disease (n =117, 25.71%), large artery atherosclerosis (n =84, 18.46%), and cardioembolism (n =63, 13.85%). Furthermore, eighteen strokes were caused by other determined etiologies (3.96%). Conclusions: Undetermined etiology was the most common etiology of AChA infarction. Hypertension, dyslipidemia, diabetes mellitus, and smoking were common risk factors for patients with AChA infarction. It is necessary to prevent the risk factors.