Primary carcinoid tumor of the kidney is an extremely rare well-differentiated neuroendocrine tumor, which is generally a low-grade malignant cancer with a good prognosis. Carcinoid tumors are rarely found in the urinary system. Here, we report a 34-year-old woman with primary renal well-differentiated neuroendocrine tumor who underwent nephron sparing surgery and no evidence of recurrence or distant metastasis was found during routine follow-up. We searched the case of renal carcinoid with the search phrase "carcinoid [title] and kidney [title]" and "carcinoid [title] and renal [title]" using the PubMed and restricted the search to articles published in English since 2013. The clinical manifestations, age, sex, tumor size, location, gross pathology, light microscopy and immunohistochemistry were analyzed. A total of 28 cases of renal carcinoid were retrieved from PubMed. Higher proportion of positive labeling of CgA, Syn, NSE and CD56 are most valuable in the diagnosis of primary renal well-differentiated neuroendocrine tumor. At present, radical nephrectomy remains the gold standard in the curative-intent therapy for well-differentiated neuroendocrine carcinoma of kidney, in metastatic renal carcinoid, long-term use of octreotide may be an effective adjuvant therapy.