Paraneoplastic syndromes are systemic, nonmetastatic manifestations associated with a variety of malignant neoplasms and occurring in a minority of cancer patients. Paraneoplastic syndromes in association with cancers of the larynx and hypopharynx are considered rare. The world literature is reviewed, dividing paraneoplastic syndromes into 4 main groups, ie, cutaneous (acanthosis nigricans, Bazex's syndrome, Bowen's disease, bullous pemphigoid, dermatomyositis, pruritus, Sweet's syndrome, yellow nail syndrome, and tylosis), endocrine (carcinoid syndrome, adrenocorticotropic hormone syndrome, Schwartz-Bartter syndrome, hypercalcemia), hematologic (Trousseau's syndrome), and neurologic (cerebellar degeneration, Eaton-Lambert myasthenic syndrome). A paraneoplastic syndrome may precede the clinical manifestations of a persistent or recurrent tumor or of asymptomatic metastases, and its onset may even, in a minority of cases, be the presenting sign of a malignancy. Otolaryngologists and head and neck surgeons should have a knowledge of the paraneoplastic syndromes in order to facilitate their detection of malignancies or recurrences.