Approximately 13 patients with hemophilia A or B have been reported to be diagnosed with acute leukemia in childhood [1, 2]. This rarity appeared again in a 13 year old male with severe hemophilia with inhibitors who presented with worsening bone pain, weight loss, and leukocytosis. Coexistence of these life-threatening disorders, hemophilia and acute leukemia, pose practical challenges in providing standard care as the former has a propensity of bleeding and the latter requires intensive treatment with systemic and intrathecal chemotherapy. Our discussion aims at highlighting critical care strategies in management of severe hemophilia and hematologic malignancies.