A 55-year-old woman was admitted for an elevated serum carbohydrate antigen-125 (CA-125) level, and a left pleural effusion, which were detected at a routine health examination. Computed tomography of the chest was performed upon admission, revealing extensive bilateral paratracheal and mediastinal lymph node enlargement with a massive left-sided pleural effusion. Subsequent analysis of the pleural fluid demonstrated consistency with an exudate, no evidence of malignant cells, and a normal adenosine deaminase. However, the pleural fluid and serum CA-125 levels were 2,846.8 U/mL and 229.5 U/mL, respectively. A positron emission tomography did not reveal any primary focus of malignancy. Finally, a surgical mediastinoscopic biopsy of several mediastinal lymph nodes was performed, revealing non-necrotizing granulomas, consistent with sarcoidosis. After a month of treatment of prednisolone, the left pleural effusion had resolved, and after 2 months the serum CA-125 level was normalized.