Abstract: The objective of this study was to describe the clinical features of Sweet syndrome in children. Our study population consisted of seven children diagnosed with Sweet syndrome over a 22-year period. Age, sex, appearance and location of lesions, associated signs and symptoms, past medical history, pathology, and subsequent disease course were documented for each patient. Fever and typical lesions were reported in most of patients in our study. The majority of patients presented with less-typical findings, such as pustules, vesicles, bullae, oral ulcerations, atrophic scars, and evidence of pathergy. Of the seven children in our study, four were found to have a preceding nonspecific upper respiratory or gastrointestinal infection, and two were diagnosed with an underlying hematologic malignancy. Our results suggest that atypical lesions are relatively common in children with Sweet syndrome and that underlying malignancy is associated with a minority of cases of pediatric Sweet syndrome.