BACKGROUND: Sweetʼs 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 Sweetʼs syndrome and whose postoperative course was complicated by encephalitis. RESULTS: A diagnosis of Sweetʼs 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 Sweetʼs syndrome are infrequently reported and have not been described previously in a patient with adenocarcinoma of the esophagus.