Paraneoplastic syndromes are extremely rare in otolaryngology.However, they can significantly influence both the therapy and outcome of patients with oncologic disease. This report deals with two cases of paraneoplastic syndromes associated with malignant tumors of the head and neck region. A 45-year-old male presented with a polymyalgia rheumatica, which was identified as a synchronous feature of a hypopharyngeal carcinoma. The symptoms disappeared after successful surgical treatment of the carcinoma. No analogous report has been published as yet. As demonstrated in a second case of a 54-year-old male, leukocytosis can be paraneoplastically associated with a squamous cell carcinoma of the tongue. The international literature is reviewed in order to analyze the incidence of different paraneoplastic syndromes related to malignancies of the upper aerodigestive tract including the rare neuroendocrine oat cell carcinoma of the larynx.