Developmental venous anomalies (DVAs) are common congenital variations of normal venous drainage that are known for their benign natural history. Isolated cases of symptomatic DVAs with associated arteriovenous (AV) shunts have recently been reported. The present case, in which thrombosis occurred in a DVA involving an AV shunt, raises intriguing questions regarding the clinical characteristics of these lesions and can be used to argue in favor of considering such lesions to be arteriovenous malformations (AVMs). A 39-year-old man presented with acute thrombosis in a complex system of anomalous hemispheric venous drainage, which included two distinct DVAs, one of which involved an AV shunt. The hemodynamic turbulences induced by a communication between shunted and normal venous outflows were the possible predisposing factor of the thrombosis. Follow-up angiographic and magnetic resonance images revealed complete recanalization of the thrombosed vessel and provided a thorough visualization of the particular angioarchitecture of the DVA. Acute thrombosis within a DVA with an AV shunt has not been reported previously and, thus, this case can be added to other reports of complications that arise in this particular type of DVA. The authors hypothesize that the presence of an AV shunt in a DVA is a risk factor for aggressive clinical behavior of the anomaly, rendering those lesions prone to complications similar to AVMs. Although no treatment can be offered, the presence of an AV shunt in a DVA warrants close follow-up observation because such lesions may represent a particular subtype of AVM and, therefore, may exhibit an aggressive clinical behavior.