Leiomyosarcomas of the head and neck is an extremely rare entity that because of its infrequency has been associated with both delayed diagnosis and misdiagnosis. Sinonasal tract is the most common site in this region. The overall prognosis is poor. It is necessary for appropriate immunohistochemical investigation for accurate diagnosis. Tonsillar leiomyosarcoma presented only 1 case in the English-language literature (PubMed, Ovid, and Proquest databases). We report a second case of leiomyosarcoma arising in the tonsil in a 38-year-old woman.
Level of evidence: Level IV therapeutic study.