Anemia is one of the characteristics of idiopathic myelofibrosis (IMF), and malignant thymoma is usually associated with various hematologic disorders, including anemia, pancytopenia, and hypogammaglobulinemia. However, the relationship between IMF and malignant thymoma has not been published before. Here, we report a 48-year-old woman who was initially diagnosed of IMF with severe anemia and transfusion dependent. Five years later, malignant thymoma was found when she was examined for chronic cough. After performing extended thymectomy, her anemia dramatically recovered to normal and sustained for 2 years till last follow-up. Her splenomegaly and myelofibrosis were also improved. We hypothesized that her malignant thymoma induced the progression of IMF, especially in anemia.