Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is a rare inflammatory disorder characterized by sudden onset of variably tender brightly erythematous-to-violaceous papules, plaques, or nodules. To clarify the frequency, clinical course, therapeutic response, and outcome of Sweet syndrome with malignancy. We conducted a retrospective review of patients diagnosed with SS between 2009 and 2019 with a diagnosis of Sweet syndrome in West China hospital. Records were analyzed for variables, clinical features, etiological factors, laboratory results, and treatment outcomes. The 25 cases under study consisted of 14 (56%) females and 11 (44%) males. Mean age was 52.2 y (range: 4-76 y). Nodules were the commonest lesion presentation (48%), involving mostly the lower limbs (52%). Pain of lesion was identified as the most common symptom (88%), followed by fever (76%) and cough (44%). Among the patients, 52% exhibited the classic form, 48% showed the malignancy-associated form, and 4% exhibited the drug-induced form in the setting of malignancy. Acute myeloid leukemia was the most common malignancy, observed in 5 of 12 patients (41.6%). Anemia (P = .002) was associated with malignancy (Chi-square test). This article is protected by copyright. All rights reserved.