Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare and inflammatory disorder characterized by fever, neutrophilia, and tender, erythematous skin lesions. The pathogenesis of Sweet syndrome involves an exaggerated immune response, with evidence suggesting a role for cytokines, particularly granulocyte colony-stimulating factor (G-CSF). Clinical manifestations vary widely, but commonly include abrupt onset of painful, raised plaques or nodules, often on the upper limbs, face, or neck. Histopathological examination reveals dense neutrophilic infiltrates in the dermis without evidence of vasculitis. Diagnosis is primarily clinical, supported by histopathology and exclusion of other similar conditions. Treatment typically involves corticosteroids, which often lead to rapid resolution of symptoms. However, relapses are common, necessitating long-term management strategies. This review aims to provide a comprehensive overview of the pathogenesis, clinical manifestations, diagnosis, and management of Sweet syndrome, highlighting recent advancements and areas requiring further research.