Thalassemia is a hematologic disorder that causes ineffective hematopoiesis and is related to severe anemia, iron overload, extramedullary hematopoiesis, and hepatomegaly. Hepatomegaly is related to significant extramedullary hematopoiesis. The other sites that are involved in extramedullary hematopoiesis are spleen, lymph nodes, paraspinal regions, kidney, pleura, and intestine, but intracranial involvement is a rare presentation. We discuss about a case with intracranial medullary hematopoiesis in a thalassemic patient.