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Mediastinal epidermoid cyst is a rare condition that is considered as a congenital disorder. Various manifestations from asymptomatic cases to serious symptoms may be found in these patients. However, some cases may be diagnosed incidentally. Here, we report an old man with recurrent pneumonia who demonstrated symptoms of respiratory distress and chest pain. The high resolution computed tomography (HRCT) and subsequent magnetic resonance imaging (MRI) showed a hypodense mass with internal calcification. CT-scan guided transthoracic needle biopsy confirmed diagnosis of mediastinal epidermoid cyst. The mass was resected through open surgery and patient’s symptoms were subsided.<br />