An 83-year-old female diagnosed with small cell carcinoma of the kidney is reported on. The clinical picture was similar to that seen with transitional cell carcinoma of the renal pelvis. A right nephroureterectomy was performed and a histological examination revealed similar morphological features to those of small cell carcinoma of the lung. Neuroendocrine differentiation was proven by a positive immunoreaction to neuron-specific enolase. A review of the literature indicated that in the urinary tract, most of the cases of small cell carcinoma occurred in the urinary bladder, with this case being the eleventh reported case of small cell carcinoma originating in the kidney. As with many of the other cases of small cell carcinoma of the urinary tract, this patient's tumor was associated with both adenomatous and squamous differentiation. The patient died 2 months after surgery, prior to any chemotherapy administration.