Résumé : PURPOSE OF REVIEW: This is a review about recent clinical developments in rare cancers of the head and neck. RECENT FINDINGS: Progress in molecular biology techniques has allowed the identification of new prognostic factors, and potential molecular-targeted therapies. This is of importance since chemotherapy continues to play a role but is still limited in this group of malignancies. New techniques of irradiation such as intensity-modulated radiotherapy and three-dimensional conformal radiotherapy appear to improve the locoregional control of these tumors. Surgery continues to be the cornerstone of treatment, with a growing interest in the technique of sentinel lymph node biopsy. SUMMARY: As salivary gland carcinomas, paranasal sinus cancers and melanoma of the head and neck are rare malignancies, these tumors must be treated in specialized anticancer centers with access to the latest surgical and irradiation techniques. Moreover, clinical studies with translational research are needed to identify strong prognostic and predictive factors, and effective molecular-targeted therapies.