Objective: Primary mitral valve (MV) tumor is a rare lesion, and to date, there have been few larger surgical series of MV tumors. We retrospectively analyzed 11 cases of primary MV tumors regarding clinical and pathological features, surgical procedure and long-term outcomes. Methods: From November 1983 to December 2008, we operated on 11 patients (age 36.3 ± 17.7 years, weight 55.4 ± 11.2 kg) with primary MV tumors. Symptoms were cardiac in 8 cases (72.7%) and neurologic in 3 (26.3%). Surgical procedures included en bloc excision and MV repair in 8 cases and tumor resection and MV replacement in 3. No radiotherapy or chemotherapy was given to patients with malignant tumors. Results: Pathological diagnosis was papillary fibroelastoma in 3 cases, myxoma in 3, lymphangioma in 1, lipoma in 1, hemangioma in 1 and sarcoma in 2. No early deaths or complications occurred. Late death occurred in 2 patients with sarcoma 1 year postoperatively. At the latest follow-up, with a maximum of 25 years (mean 10.6 ± 8.8), the 9 survivors were in New York Heart Association functional class I with normal MV function and no echocardiographic evidence of local recurrence. Conclusions:The majority of primary MV tumors are benign. They can cause cardiac or neurologic symptoms and should be excised as soon as a diagnosis is made. For benign tumors, valve-sparing resection and valve repair are often possible with excellent long-term outcomes. The prognosis of malignant MV tumors is poor.