Cancer in the oral cavity and oropharynx is rare yet lethal; at least 50% of affected patients die of this disease. This health condition is associated with tobacco exposure and alcohol consumption, although there is a growing incidence among non-smoker/non-drinker populations, especially young people, in which human papillomavirus infections seem to be another cause. Unfortunately most of these patients are diagnosed in advanced stages, in spite of the accessible location of the tumors. Tobacco control and accurate diagnosis of early and premalignant lesions are crucial in order to diminish incidence and prescribe efficient treatments aimed at achieving the best local control, with minimal functional and aesthetic impact and better survival. Although there is some controversy regarding the current treatments, for advanced tumors the best results are obtained with multidisciplinary management; however, the results are still poor. We present an updated review of the information available on this deadly, but barely known disease.