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Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by palpable purpura, arthralgia or arthritis, GI symptoms, and renal involvement. Superior mesenteric artery (SMA) syndrome, a rare condition, occurs when the third part of the duodenum is compressed between the aorta and the SMA, leading to upper intestinal obstruction. This case report describes the clinical presentation, diagnostic process, and management of an eight-year-old girl with HSP complicated by SMA syndrome. The patient initially presented with abdominal pain and vomiting, eventually developing the characteristic rash of HSP. While initial management was supportive, her condition deteriorated. Treatment with intravenous methylprednisolone resulted in significant symptom improvement and resolution of both SMA syndrome and HSP manifestations. This case highlights the need to recognize SMA syndrome as a potential complication of HSP and demonstrates the effectiveness of steroid therapy in managing this condition. Further research is needed to develop comprehensive treatment guidelines for HSP patients with SMA syndrome., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Shayah et al.)