Pyeloureteritis cystica is a rare cystic disease formed in the mucosa or submucosa of the renal pelvis and ureter caused by chronic inflammation and irritation, and characterized by multiple filling defects noted on the contrast imaging. The clinical course is usually slow, but manifests if the lesionscause infection or obstruction. Stones are further complication to the disease. We report a case of 69-year-old woman with right flank pain, who had an pyeloureteritis cystica withnonfunctioning kidney caused by proximal ureter stone. The diagnosis was made after nephroureterectomy, followed by pathologic examination.