Brain arteriovenous malformations (bAVMs) are associated with a high risk of intracerebral hemorrhage, which causes severe complications in patients. Although the genetic factors leading to hereditary bAVMs have been extensively investigated, their pathogenesis are still under study. This review examines updated data on the molecular and genetic aspects of bAVMs, the architecture of microvasculature, the roles of angiogenic factors, and signaling pathways. The compiled information may help us understand the pathogenesis of both sporadic and hereditary bAVMs and develop appropriate preemptive treatment approaches.