We report a case of CA19-9-producing transitional cell carcinoma of the renal pelvis. A 59-year-old male patient with left hydronephrosis was referred to us from a local physician. Retrograde pyelogram revealed irregular filling defects involving calices, pelvis and proximal ureter. The serum CA19-9 level was elevated. Under the diagnosis of renal pelvic tumor, we performed radical left nephroureterectomy. The tumor was histologically diagnosed as transitional cell carcinoma. The tumor cells showed positive immunostaining for CA19-9. The serum CA19-9 level was normalized after the operation. To our knowledge, this is the 28th case of a CA19-9-producing tumor in the Japanese literature.