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The present report aimed to present a unique case of idiopathic retroperitoneal fibrosis (RPF) presenting features of unilateral ureteral and sigmoid colon obstruction. RPF is a rare disorder with unclear etiology.A 43-year-old female had a 10-day history of lower right abdominal and lumbar pain. Gynecological examination, ultrasound, and computed tomography (CT) were all suggestive of right ovarian tumor. An enhanced CT showed right-sided hydronephrosis. The patient was diagnosed as having ovarian cancer. Ten days after hospitalization, a right intraureteral stent with a double-J catheter was inserted. Upon exploring the abdomen, unyielding RPF was encountered. A partial sigmoidectomy and colostomy were performed. Postoperative pathological results suggested idiopathic RPF. She received steroid treatments.RPF is a rare disease that can be misdiagnosed. Our understanding about its presentation has to be improved and it should be considered as a differential diagnosis for patients presenting with abdominal diseases.