Objectives: Cerebral venous thrombosis (CVT) is a disease with varying clinical presentation and diagnosis presents many challenges in clinical practice. We investigated, whether D-dimer levels reflect clinical presentation, radiologic features, and outcome in CVT. Methods: We included all consecutive patients with CVT treated in our hospital from 1987 to 2010 with D-dimer levels measured before initiation of anticoagulant treatment. D-dimer was categorized as low (<0.5 mg/L), intermediate (0.6-2.0 mg/L), and high (>2.0 mg/L). Based on delay from symptom onset to hospital presentation mode of onset was categorized as acute (<2 days), subacute (2-14 days), or chronic (>14 days). Results: In 71 patients included median level of D-dimer was 1.40 mg/L (range 0.05-13.0 mg/L). In 9 (12%) patients D-dimer was low, and of these, 7 presented with subacute and 2 with chronic mode of symptom duration. Elevated D-dimer levels were associated with thrombosis in multiple sinuses (P = 0.044). Longer symptom duration was correlated with low D-dimer levels (P = 0.010). Conclusions: In clinical practice, low levels of D-dimer cannot rule out CVT in patients with subacute or chronic disease. High D-dimer levels correlate with greater thrombus extension and acute onset of symptoms. (c) 2013 Elsevier B.V. All rights reserved.