Background: The basal ganglia, an important functional integrity of the brain, comprises multiple subcortical nuclei. The area is mainly supplied by the perforating branches of ipsilateral middle cerebral artery (MCA). The unilateral non-traumatic basal ganglia hemorrhage, commonly located in the putamen, most often results from hypertension.Method: Simultaneous bilateral basal ganglia hemorrhage (SBBGH) is extremely rare. There have been less than 30 case reports worldwide so far. Our article is the first comprehensive review on SBBGH. Conclusion: The clinical features, causes and prognosis were summarized and discussed, in perspective of providing guidance for future diagnosis and treatment.