We treated a patient with nongranulomatous panuveitis associated with idiopathic retroperitoneal fibrosis who had symptoms of abdominal pain, severe fever, leg edema, and blurred vision. A high C-reactive protein level, a high erythrocyte sedimentation rate, anemia, and abnormalities of the immune system were seen. Retrograde pyelography disclosed persistent ureteral obstruction. A computed tomographic scan and magnetic resonance imaging showed a dense plaque of fibrous tissue around the kidney and hydronephrosis. Biopsy disclosed fibrosis infiltrated with lymphocytes, plasma cells, histiocytes, and eosinophils. A moderate degree of cellular inflammation in the anterior chamber and cystoid macular edema were present at ocular examination. We believe idiopathic retroperitoneal fibrosis to be a new cause of uveitis of suspected autoimmune origin.