Cerebral small vessel disease refers to a group of pathological processes, neuroimaging features, and clinical symptoms, with various etiologies that affect the small arteries, arterioles, venules, and capillaries of the brain. The onset of cerebral small vessel disease can be insidious. It has various symptoms, some of which can attack acutely. Acute cerebral small vessel disease is characterized by lacunar stroke and brain parenchymal hemorrhage. The latter mainly includes hypertensive hemorrhage and cerebral amyloid angiopathy. This article summarizes the research advances of acute cerebral small vessel disease from the aspects of pathogenesis, clinical manifestations, neuroimaging features, and treatment methods, discussing characteristics and clinical challenges.