Developmental venous anomalies (DVAs) are the most common slow-flow venous malformation in the brain. Most DVAs are benign. Uncommonly, DVAs can become symptomatic, leading to a variety of different pathologies. DVAs can vary significantly in size, location, and angioarchitecture, and imaging evaluation of symptomatic developmental venous anomalies requires a systematic approach. In this review, we aimed to provide neuroradiologists with a succinct overview of the genetics and categorization of symptomatic DVAs based on the pathogenesis, which forms the foundation for a tailored neuroimaging approach to assist in diagnosis and management.ABBREVIATIONS: BRBNS ¼ blue rubber bleb nevus syndrome; CCM ¼ cerebral cavernous malformation; CMMRD ¼ constitutional mismatch repair deficiency syndrome; CVMS ¼ cerebrofacial venous metameric syndrome; DVA ¼ developmental venous anomaly; WMH ¼ white matter hyperintensities D evelopmental venous anomaly (DVA) is an extreme varia-