We report a rare case of leiomyosarcoma arising in the nasal cavity. A 45-year-old woman visited our hospital because of recurrent epistaxis in 1998. A biopsy was performed on a mass in the posterior area of the left nasal cavity, and a histological diagnosis of leiomyosarcoma was made. Although the nasal tumor was resected via left lateral rhinotomy, tumor recurrence was detected. In addition, multiple metastatic tumors were detected in both lungs. Therefore, CyberKnife therapy was performed for the recurrent tumor and radiofrequency ablation for a large tumor among the metastatic lung tumors. Although the patient died of multiple metastasis of the whole body in 2007, tumors in the lungs and the primary site, which were treated with radiofrequency ablation and CyberKnife therapy, did not increase. Leiomyosarcoma has a poor prognosis, and when recurrence or distant metastasis occurs, radical cure is even more difficult. In this patient, CyberKnife therapy and radiofrequency ablation were effective for treating local recurrent and metastatic lesions. Even in patients in whom a radical cure is difficult due to recurrence or distant metastasis, CyberKnife therapy and radiofrequency ablation can relieve symptoms and prolong survival.