The xanthofibrosarcoma is a malignant variant of fibrous xanthoma. It is a rare tumor that derives from histiocytes. A case believed to be the first xanthofibrosarcoma reported in the larynx is presented. Differentiation between this tumor and a benign lesion was difficult. The tumor proved malignant by metastasizing.
Although few fibrous xanthomas become malignant they should raise a high index of suspicion when they are large, have infiltrative margins, and tend to recur locally. Treatment is by wide surgical excision. Close follow-up is mandatory. The prognosis once metastases appear is extremely poor.