Brain arteriovenous malformations (bAVMs) are a complex congenital cerebrovascular disease. The lack of common capillaries results in blood flowing directly from the arteries to the veins, forming abnormal vascular malformations between the arteries and veins. BAVMs are usually found due to "epilepsy, intracranial hemorrhage, and focal neurological dysfunction", with a low incidence rate but a high mortality and disability rate. However, the specific pathogenesis of bAVMs is not fully understood. This article reviews the signaling pathways, pathophysiological mechanisms, and non-coding RNAs related to the pathogenesis of bAVMs, with the aim of providing new diagnostic and therapeutic strategies for bAVMs.