Background: Malignant schwannomas are rare malignant mesenchymal tumors often associated with neurofibromatosis. They occur less frequently in the head and neck than in other regions. Patient: A case history of a primary malignant schwannoma of the head and neck area in a 27-year-old man is reported. The tumor was located in the left submandibular region. The patient did not have any functional deficits. The tumor was totally removed. There have been no signs of either recurrence or metastasis within the two years following diagnosis and surgery. Discussion: The microscopic and immunohistochemical findings are presented, and the importance and therapy of this very rare malignant tumor of the head and neck area are discussed. Conclusion: Malignant schwannoma in the head and neck region is rare. Radical resection is the treatment of choice.