Objective To report 2 cases of artery of Percheron (AOP) infarction and conduct a literature review of 86 cases of AOP infarction, summarizing the clinical characteristics of the disease. Methods The clinical diagnosis, treatment and prognosis of 2 patients with AOP infarction who were treated in The Frist Affiliated Hospital of Xi'an Medical University were reported. The data of 86 cases of AOP infarction were obtained from the literatures at home and abroad. All the clinical data were statistically analyzed. Results A total of 88 AOP patients were enrolled, including 41 males (46.6%) and 47 females (53.4%), with an average age of (58.7±18.9) years old, ranging from 6 to 93 years old. The etiology of acute ischemic stroke was classified according to the Trial of Org 10172 in Acute Stroke Treatment classification:small artery occlusion in 23 patients (26.1%), cardiac embolism in 22 patients (25.0%), other cause in 22 patients (25.0%), and unknown cause in 21 patients (23.9%). There were 80 cases (90.9%) without intravenous thrombolysis and 83 cases (94.3%) with acute onset. There were 80 cases (90.9%) with consciousness disorder, 41 cases (46.6%) with ophthalmoplegia, 37 cases (42.0%) with speech disorder (dysarthria or thalamic aphasia), 34 cases (38.6%) with limb motor dysfunction, 34 cases (38.6%) with abnormal pupils, 15 cases (17.0%) with dizziness, 14 cases (15.9%) with memory loss, 5 cases (5.7%) with ataxia, and 3 cases (3.4%) with facial nerve paralysis. The most common infarction sites were simple thalamic infarction (44 cases, 50.0%), followed by thalamic infarction combined with mesencephalic infarction (35 cases, 39.8%). The typical midbrain "V" sign was found in 14 cases (15.9%). After treatment, 73 cases (83.0%) were improved, 11 cases (12.5%) died, 2 cases (2.3%) had recurrent infarction, and 2 cases (2.3%) had no change. The age and infarction site were related to the prognosis of patients (both P<0.01). Conclusion The early symptoms of AOP infarction are variable. Diffusion weighted imaging in the acute phase suggests that high signals can be seen in the thalamus and/or midbrain, and there may be a typical "V" sign in the midbrain. Early AOP infarction can be treated with intravenous thrombolysis and other ischemic stroke treatments. The prognosis is related to the age and infarction site.