我們報告一例第一型腎小管酸中毒之病例，此病患出現之臨床表徵為經常無症狀地排出結石，同時放射線檢查呈現兩側腎臟有實質鈣化現象，經由新陳代謝評估，包括血液、尿液生化檢查及酸負荷實驗，診斷為腎小管酸中毒合併尿路結石症，並討論其病因學，治療方法及預防。

A case of renal tubular acidosis, type 1 (RTA-1) is presented; the patient passed urinary stones frequently without other symptoms. Plain abudominal x-ray revealed extensive renal parenchymal calcification in both kdneys. Metabolic evaluation including serum, urine biochemistry and acid loading tests were done. A diagnosis of RTA with urolithiasis was established, and the etiology, treatment and prophylaxis are discussed.