A 12-year-old boy who presented with headache and papilledema was diagnosed with cerebral sinus venous thrombosis and promyelocytic leukemia. Cerebral sinus venous thrombosis is an uncommon manifestation of acute promyelocytic leukemia, and acute promyelocytic leukemia is an unusual etiology for cerebral sinus venous thrombosis. This case highlights the importance of a complete evaluation for risk factors in a child presenting with cerebral sinus venous thrombosis. Anticoagulation was initiated for treatment of cerebral sinus venous thrombosis, and new asymptomatic hemorrhage was identified in the cerebellum 1 day later. Anticoagulation was discontinued for 5 days and then reinitiated in the intensive care unit without hemorrhage expansion or other complications. We review the literature regarding anticoagulation in children with cerebral sinus venous thrombosis and issues specific to acute promyelocytic leukemia. This case suggests that in a controlled setting, anticoagulation may be safe in selected patients with cerebellar hemorrhage.