Leiomyosarcoma (LMS) accounts for approximately 7% of all soft tissue sarcomas and occurs most frequently in the gastrointestinal tract and uterus. LMS of the nasal cavity and paranasal sinuses are rare and only about 40 cases of LMS of the nose and paranasal sinuses have been reported in the literature. A further case of LMS of the right turbinate is presented, treated twice by surgery. To date, the patient has been free of disease for more than 48 months. The symptoms, pathology, treatment and prognosis, and origin of this tumor are discussed. Initially, LMS should be treated by extensive surgical excision, but long-term follow-up is essential due to the high rate of local recurrence. Radiotherapy and chemotherapy are insufficient therapeutic approaches. Frequency of recurrence and prognosis depend on the tumor site. The prognosis of LMS of the nasal cavity is better than LMS of the paranasal sinuses.