Sweet syndrome (acute febrile neutrophilic dermatosis) is a disorder of unknown etiology. It has been associated with autoimmune processes, malignancies, infections, drug reactions, and gastrointestinal disorders such as inflammatory bowel disease. We describe the case of a 51-year-old man who presented with severe pain in his tongue and throat and referred pain in his right ear, along with odynophagia, fever, and hoarseness of 48 hours'duration. An oral and oropharyngeal examination revealed the presence of aphthous ulcerations, as well as a 3 x 3-cm raised inflammatory lesion on the right anterior lateral tongue and a 5 x 5-mm bulla on the hard palate in the midline. In addition, erythematous papules and macules were noted on his face, neck, and extremities. Cultures, a biopsy, and laboratory tests yielded a diagnosis of Sweet syndrome. The patient was prescribed oral prednisone, and his signs and symptoms resolved within 2 months. Although Sweet syndrome is uncommon, even in dermatology practice, its head and neck and oral manifestations and its association with paraneoplastic disease warrant the need for otolaryngologists to be aware of the condition.