We report a rare case of sarcoidosis of the ureter in a 65-year-old Japanese man. Left nephroureterectomy and regional lymph node dissection were performed under the clinical diagnosis of transitional cell carcinoma of the left ureter with lymph node metastasis. Microscopically, noncaseous epithelioid granuloma with large Langerhans cells was noted in the ureter and dissected lymph nodes. Pulmonary lesions were not found on computed tomography. The final diagnosis was sarcoidosis of the ureter. Although sarcoidosis is rare in the genitourinary tract, it should be considered in the differential diagnosis of urologic conditions.