Twenty-eight cases of pyeloduodenal fistula are reviewed from the standpoint of pathogenesis, clinical presentation, diagnosis, and management. Our review suggests the majority of these fistulas involved the right kidney and occurred as a result of chronic renal inflammatory disease. The fistulas are most effectively diagnosed with retrograde pyelography. The review suggests the best management of pyeloduodenal fistula is nephrectomy and primary closure of the duodenum.