Pure red cell aplasia is an uncommon cause of anaemia rarely associated with thymoma. A combination of immunosuppressive therapy and thymectomy offers a potential cure. Thymectomy alone rarely results in anaemia resolution. A seventy-three-year-old male with Klinefelter syndrome presented with progressively increasing shortness of breath and anaemia. Serological testing supported primary bone marrow pathology, and a bone marrow biopsy was performed. A pure red cell aplasia was seen on bone marrow examination, and computed tomography of the chest demonstrated a thymoma. Thymectomy was performed, and histology revealed a thymolipoma. Complete anaemia resolution was achieved following thymectomy alone. This suggests that thymomas may directly mediate immune dysregulation resulting in erythroid precursor destruction.