We hereby present the exceptional case of hypoglycemic coma associated with a giant benign localized fibrous tumor of the pleura (LFTP). A 79-year-old woman was found to have a small peripheral nodular lesion in the right hemithorax 7 years ago. A fine needle aspiration biopsy provided a diagnosis of LFTP, but the patient refused surgery. Six years later hypoglycemic coma and respiratory insufficiency appeared in association with a giant mass completely filling the right chest and shifting the mediastinum contralaterally. The tumor was completely resected, and complete relief of the hypoglycemic and respiratory symptoms was achieved. The patient is in good metabolic and respiratory conditions and disease-free 14 months after the operation.