BACKGROUND Chronic immune thrombocytopenia purpura (ITP) is associated with a higher incidence of adverse outcomes, increased morbidity and mortality rates, and higher health care costs, especially in open-heart surgery. The information regarding managing chronic ITP in patients undergoing mitral valve replacement (MVR) surgery is scarce, and reported cases are limited. CASE REPORT A 42-year-old woman with more than 20 years of history of immune thrombocytopenia purpura (ITP) had episodes of breathing difficulties in the last 4 years. The patient was diagnosed with severe mitral stenosis (MS) and moderate mitral regurgitation (MR). Laboratory examination before surgery showed thrombocytopenia (49 000/µL). Therefore, the surgery was postponed until the platelet count exceeded 100 000/µL. The patient was given 10 units of thrombocyte concentrate 1 day before surgery and 500 mg of methylprednisolone 3 times a day orally for 5 days as preoperative management. Under a total cardiopulmonary bypass, MVR was performed using a bioprosthetic valve. Postoperative transthoracic echocardiography (TTE) showed no valvular leakage in the surrounding of the prosthetic valve and that the valve was functioning normally. Platelet monitoring was conducted, and the platelet count increased to 147 000/µL on the third day. CONCLUSIONS Our case report shows that aggressive preoperative platelet count correction and treatment may lower the risk associated with a low and unstable platelet count and reduce the risk of mortality and morbidity in patients with ITP who undergo MVR procedures.