Abstract
Spindle cell carcinoma is a rare neoplasm of the upper respiratory tract which occurs in adults, most commonly in the larynx. In the literature only one case of spindle cell carcinoma, located in the maxilla, has been reported in a child. We report the first presentation of a spindle cell carcinoma in a child, which was located in the parotid gland, together with the clinical course. The diagnostic challenge associated with this unusual disorder is elucidated, as well as the role of immunohistochemical and cytogenetic examination to define the nature of these lesions.