We present 28 patients with basal ganglia ischemic stroke and describe the main neurological manifestations, neuroimaging findings, risk factors, and outcome. In 23 cases, at least 1 risk factor was identified. A total of 7 cases (25%) had antecedent of varicella infection and 7 cases (25%) had preceding mild head trauma. Similar antecedents were present only in 2.6% and 5.3% of patients with nonbasal ganglia stroke, respectively (odds ratio: 12.2, 95% confidence interval: 2.04-124.65 and odds ratio: 5.92, 95% confidence interval: 1.32-29.7). The arterial abnormalities identified in 10 patients were narrowing (6) or occlusion (4) of the M1 segment. After a median follow-up of 24 months, 19 patients had a good outcome. Magnetic resonance angiography and catheter cerebral angiography played an important role in the identification of arterial disease. We propose that basal ganglia infarction is a different group of ischemic stroke with prevalent risk factors (varicella infection and mild head trauma) and good outcome.