Hemophilic pseudotumor is a rare but well-known complication of hemophilia manifesting as recurrent hemorrhage and progressive enlargement of hematoma. A patient with severe hemophilia has 1% to 2% chance to develop pseudotumor. The chronic pressure of osseous hemorrhage usually results in bone destruction or resorption. Cranial hemophilic pseudotumors are extremely rare, with only 7 reported cases associated with mild or moderate factor VIII or IX deficiency. A 42-year-old man with a mild factor VIII deficiency developed a pseudotumor of the bilateral skull. Computed tomography and magnetic resonance imaging revealed an extra-axial lesion with bone destruction, and signal changes are consistent with chronic hemorrhage. With adequate factor-deficient replacement therapy, surgical removal was performed. Histologic examination disclosed old blood coagulum. No recurrence was observed in 3 years of follow-up. Cranial hemophilic pseudotumor is extremely rare, and with adequate factor-deficient replacement therapy, surgical management is a safe and effective way for cranial hemophilic pseudotumor treatment.