Rosai-Dorfman disease is a rare condition with poorly understood pathogenesis at this time. Although it often involves the lymph nodes, it can present nearly anywhere at extranodal sites. Patients are frequently asymptomatic, but surgical debulking is currently the only method of treatment that has shown benefit for patients requiring intervention. This case report discusses a unique presentation of Rosai-Dorfman disease involving the ureter of a 49-year-old woman with known history of sarcoidosis, discovered incidentally on routine CT scan.
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