The posterior fossa arteriovenous malformation (AVM) is uncommon and different from other intracranial AVM in its natural history, diagnosis, treatment, prognosis, and other features. Authors present a review of the actual procedures of diagnosis and treatment in the field of infratentorial cerebral arterio–venous malformations, based on the actual literature data. Pre–therapeutic considerations, such as clinical presentation and diagnostic evaluation, are initially discussed because they are crucial in choosing the optimal treatment. Posterior fossa AVMs merit multimodality intervention when feasible in most cases because of their higher risk of rupture and higher potential of morbidity and mortality. In addition, we present two significant cases treated in our department.