A case of pulmonary varices in a 24-year-old man with mitral regurgitation is reported. A brief summary of the literature on pulmonary varices associated with mitral valve disease is presented, with special reference to its etiology and surgical treatment. It is strongly suggested that production of varicosities of the pulmonary vein requires not only increased pulmonary venous pressure but also other local factors. If the patient has symptoms due to mitral regurgitation, operation for mitral valve disease is indicated. Direct operative intervention for the varices should not be performed.