Acute neutrophilic dermatosis, first described in 1964 by Robert Douglas Sweet, is characterized by sudden onset fever, neutrophilic leukocytosis, and well demarcated erythematous papules, nodules, and plaques with dense neutrophilic infiltrates on histologic evaluation.Here is a report of a 7-year-old girl who presented with high grade fever, and discrete erythematous papular skin eruptions, which gradually increased in number and involved the face, trunk, extremities, palms, soles, hard palate, and palatal tonsils. The skin eruptions evolved to pustules and after coalescing caused large crusted plaques, with mild tenderness but without any pruritus. White blood cells were 36900/ml with 92% neutrophils. Skin biopsy test was compatible with acute febrile neutrophilic dermatosis, so prednisolone (1 mg/kg/day) was started that led to a rapid defervescence and significant improvement of dermatosis. After a few days, the patient presented with fever and arthritis of right elbow, both ankles, and wrists, so she was re-admitted. She also developed bloody diarrhea during the hospital stay. Colonoscopy and intestinal biopsy were performed, which confirmed the diagnosis of ulcerative colitis. Prednisolone, sulfasalazine, and naproxen were prescribed. The fever and diarrhea stopped after a few days and joint swelling decreased. She was discharged 2 weeks after the admission with a rather good general condition. Inflammatory bowel disease can be one of the several conditions accompanying sweet syndrome.