Background  Sweet's syndrome, also known as febrile neutrophilic dermatosis, can occur in patients with an underlying malignancy and can present with extracutaneous manifestations, including neurologic symptoms.

Methods  This report describes a 62-year-old man with adenocarcinoma of the esophagus who developed Sweet's syndrome and whose postoperative course was complicated by encephalitis.

Results  A diagnosis of Sweet's syndrome with neurologic manifestations was made, and the patient was treated with oral corticosteroids. His symptoms improved markedly within 12 h.

Conclusion  Neurologic symptoms in Sweet's syndrome are infrequently reported and have not been described previously in a patient with adenocarcinoma of the esophagus.