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Primary neuroendocrine carcinoma of the kidney is a rarely observed clinical condition because neuroendocrine cells are not found in kidney parenchyma. It’s not clinically and radiologically possible to distinguish from other kidney tumors. Incidence with horseshoe kidney anomaly, it should be considered as a definitive diagnosis for the patients with this condition. In this case report, we reported about a carcinoid tumor in horseshoe kidney in a 37-year-old woman.
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