Establishing the diagnosis of cerebral venous thrombosis (CVT) can often be challenging to physicians. CVT presents with variable symptoms, resulting in delay in establishing the correct diagnosis and treatment. This chapter provides practical recommendations on the currently available diagnostic and treatment options in patients with suspected acute CVT. The diagnostic value of computed tomography, magnetic resonance imaging, and catheter angiography are also discussed. This chapter also includes recommendations for medical treatment with systemic anticoagulation, the role of endovascular therapy, and indications for surgical interventions including hemicraniectomy and control of intracranial pressure. Formal evidence-based guidelines released by the American Heart Association in 2011 are also outlined in this chapter.