Developmental venous anomalies (DVAs), formerly known as venous angiomas, have become the most frequently diagnosed intracranial vascular malformation. DVAs are currently considered congenital cerebrovascular anomalies with mature venous walls that lack arterial or capillary elements. They are composed of radially arranged medullary veins, which converge in an enlarged transcortical or subependymal collector vein, and have characteristic appearances (caput medusae) on magnetic resonance imaging and angiography. DVAs were once thought to be rare lesions with substantial potential for intracerebral hemorrhage and considerable morbidity. The prevalence of incidental and asymptomatic DVAs has been more apparent since the advent of magnetic resonance imaging; recent cohort studies have challenged the once-held view of isolated DVAs as the cause of major neurological complications. The previously reported high incidence of intracerebral hemorrhage associated with DVAs is currently attributed to coexistent, angiographically occult cavernous malformations. Some patients may still have noteworthy neurological morbidity or die as a result of acute infarction or hemorrhage directly attributed to DVA thrombosis. DVAs can coexist with cavernous malformations and arteriovenous malformations. Such combination or transitional forms of malformations might suggest common pathways in pathogenesis. Recent data support a key role for DVAs in the pathogenesis of mixed vascular malformations.