Sarcomatoid carcinoma of the renal pelvis is a rare clinical entity. To the best of our knowledge, only 14 cases of this type of neoplasm have been reported in the literature to date. In the present study, the records at The First Affiliated Hospital, Medicine School of Zhejiang University (Hangzhou, Zhejiang, China) between 2000 and 2010 were reviewed to identify patients with primary renal pelvis sarcomatoid carcinoma (RPSC). A particular emphasis was placed on the treatment, recurrence and survival outcome. Eight patients with RPSC were identified and treated with nephrectomy or nephroureterectomy. All of the patients presented with Grade 3 RPSC. According to the TNM classification system, 2 patients were in stage pT2, 5 in stage pT3 and 1 in stage pT4. Adjuvant chemotherapy was administered to four patients, and the mean follow-up period was 27.5±41.0 months. In total, 6 patients succumbed to the disease with a mean survival time of 7.7±5.3 months (range, 1–18 months), while 2 patients were free of disease at 54 and 120 months, respectively, following treatment. The mean disease-specific survival time was 27.5±41.0 months and the 1-year recurrence-free survival, 1-year survival and overall survival rates were 37.5, 37.5 and 25%, respectively. The present analysis suggests a poor prognosis for the majority of RPSC patients, most likely resulting from the advanced stage of the disease at diagnosis and a poor response to systemic therapy. To improve the survival rate of RPSC, it is therefore essential to perform an early diagnosis and early radical surgery. Intravesical instillation is not essential following surgery.