Abstract: We report on an 18-month-old boy with a typical presentation of Sweet syndrome following an upper respiratory tract illness. No evidence of hematologic malignancy was found and he was successfully treated with oral prednisolone. A comprehensive literature review has summarized the features of pediatric Sweet syndrome from 66 reported cases. Cases occurring under the age of 3 years had a male predominance and no association with malignancy, whereas cases over the age of 3 years had an equal sex distribution and were strongly associated with myeloid line hematologic malignancies. Identified complications were serious with an overall mortality of 9% rising to 40% with cardiovascular involvement.