Myxomas rarely are located on the mitral valve. We report the case of a 44-year-old man operated on successfully with mitral valve conservation, which is the 21st case of mitral valve myxoma reported in the western literature. Among the cases reported in the literature, the diagnosis was made at the time of autopsy in 6 cases, with premortem heart failure in 1 case. A clinical diagnosis was made in 15 cases on the basis of the following symptoms: peripheral embolism in 9 cases, cardiac signs in 4 cases, and constitutional manifestations in 2 cases. The clinical presentation of mitral valve myxoma differs slightly from that of other cardiac myxomas in that it has a lower incidence of constitutional manifestations. The gold standard for noninvasive diagnosis and localization of cardiac myxomas is transesophageal echocardiography. It allows for the detection of early, small valvular tumors and may help to characterize better their location and echostructure, facilitating the choice of an optimal surgical approach through preoperative study of the integrity and mobility of the valve. Conservative operative treatment by resection of the area of implantation of the myxoma followed by suture repair of the valve and annuloplasty may be recommended as the most appropriate treatment option for this rare condition.