Cardiac myxomas are the most common primary tumors of the heart. Although these tumors have been reported in all cardiac chambers, myxomas arising from heart valves are extremely rare. Here, the details are described of a patient with mitral valve myxoma, and a review is provided of 99 cases reported in the literature. Mitral valve myxomas most often occur in middle-aged patients, with a slight female predominance. Most of the tumors arise in an isolated fashion on the atrial side of the anterior mitral valve leaflet. Embolic events were the most frequent manifestation, followed by obstructive symptoms. Unlike atrial wall myxomas, these tumors have a lower incidence of constitutional manifestations. Tumor resection with valve preservation is the usual method of surgical treatment in isolated tumors, while most patients with multiple valve myxomas underwent valve replacement. Mitral valve myxomas should be considered in the differential diagnosis of mitral valve tumors, with an awareness of a relatively high risk of embolic events. This location should not raise suspicion for a heritable myxoma syndrome. Prompt surgical resection is warranted to reduce complications.