Mitral valve is a complex cardiac structure whose function depends on a proper synchronization between each mitral valve apparatus. Considerable headway over the years has been made toward unraveling the theoretical aspects which unifies the dynamic function, structural properties, and pathobiological, pathophysiological performance of the cardiac valves. However, the aspect of mitral valve regurgitation caused by left atrial myxoma still remains a gray area in the field as the mechanism(s) behind the masked and/or the resultant mitral valve regurgitation in relation to left atrial myxoma remains elusive. Although the regurgitations in most scenarios are masked due to the presence of the myxoma itself, however, both invasive and noninvasive techniques employed cannot ascertain if the regurgitation seen is a resultant or concomitant factor as the underlying pathological processes causing mitral valve regurgitation play key roles in the disease pathology as it could be a series of activated measures caused by the myxoma itself. Elucidating the role left atrial myxoma plays in mitral valve regurgitation is critical to improving our understanding as the aim of this chapter is to discuss the current knowledge of mitral valve regurgitation caused by left atrial myxoma in succinct with the elusive underlying pathological sequential cascade activated due to the presence of this neoplasm.