Isolated or predominantly right upper pulmonary edema is an atypical and not well-known finding in mitral insufficiency. In systole, during regurgitation from the left ventricle to the left atrium, targeting of blood selectively at the orifice of the right superior pulmonary vein increases the local development of edema. A 62-year-old male patient had been admitted previously for acute dyspnea, cardiomegaly and right pleural effusion and diagnosed with congestive heart failure after improvement on diuretic treatment. Two weeks later, he was readmitted for bilateral upper lobe but predominantly right upper lobe consolidation in addition to orthopnea, cough and white frothy sputum production. These consolidations resolved completely, and the clinical findings of left ventricular failure improved within 24 hours on diuretic treatment. Owing to the supporting history, clinical and radiological findings above, and the determination of mitral insufficiency and regurgitation on echocardiographic examination, the case was considered to be a predominantly right upper lobe pulmonary edema and was presented by reviewing the pertinent literature.