Mediastinal liposarcoma is an extremely rare malignancy with a poor prognosis. We report the case of a 73-year-old man who presented with severe respiratory distress. He had previously been diagnosed with mediastinal liposarcoma, which had been treated with extirpation 6 years ago. The histological type was a well-differentiated liposarcoma. Three years ago, he experienced respiratory distress due to postoperative recurrence of mediastinal liposarcoma. To establish an airway, tracheotomy was urgently performed. Tracheal stenosis also developed because the sarcoma progressed to the trachea after tracheostomy. We performed radical resection of the recurrent mediastinal liposarcoma combined with the resection of the laryngopharynx and cervical oesophagus and reconstruction using a free jejunal graft. We then performed anterior mediastinal tracheostomy with a pedicled omental flap. There has been no recurrence in the 3 years since the last operation.