Purpose: To present a rare clinical presentation of renal pelvis small cell neu-roendocrine carcinoma. Case report: A 58-year-old female visited our urology de-partment due to painless gross hematuria for two weeks. A renal ultrasound revealed right-side hydronephrosis with a mass lesion on the renal pelvis. Enhanced comput-erized tomography demonstrated a 3 cm localized enhanced mass on the right renal pelvis causing right hydronephrosis. A right nephroureterectomy with bladder cuff resection was performed. Microscopically, the tumor cells showed typical features of small cell neuroendocrine carcinomas, such as relatively small cells, a high N:C ratio, poorly defined cell borders, and scant cytoplasm. Immunohistochemical stud-ies revealed that the tumor cells were positive for cytokeratin and GATA-3. Neuro-endocrine differentiation demonstrated positive staining for synaptophysin, chro-mogranin A, and CD56. The patient was referred to the medical oncology depart-ment for adjuvant chemotherapy. Follow-up imaging at 9 months showed no signs of local recurrence or distant metastasis. Conclusion and Importance: There is no definite radiologic sign to differentiate renal pelvis small cell neuroendocrine carci-noma from other kidney tumors preoperatively at present due to limited case reports. Once diagnosed, postoperative adjuvant therapy is mandatory due to its vigorous growth pattern.