Postoperative respiratory distress and pulmonary edema can be seen after a wide variety of serious clinical situations, or rare diseases such as familial Mediterranean fever (FMF). FMF is a multisystemic disorder characterized by recurrent bouts of fever and pain due to inflammation of the peritoneum, synovia, or pleura. We report a case with history of FMF who developed postoperative respiratory distress after repairing the abdominal incisional hernia. Ten hours after administration of colchicine, the patient's symptoms were reduced. This rare disease should be included as a differential diagnosis for acute-onset respiratory distress in postoperative period.