Neuroendocrine tumors(NET) originating in the kidney account for less than 1% of renal epithelial malignancies, and are rarer when combined with horseshoe kidney. This article reported a patient of NET with horseshoe kidneys. The patient was admitted to hospital because of the pain of left waist and back for more than 1 month. Enhanced CT examination showed that horseshoe kidney, left kidney mass, about 5.0 cm × 4.0 cm, and about 2 cm from the gorge. There was a separate blood supply in the gorge. The patient underwent laparoscopic radical left nephrectomy. Intraoperatively the canyon was separated, and the tumor and left kidney were completely removed. The postoperative pathological results was NET. No auxiliary treatment was given after surgery. After 15 months of follow-up, no tumor recurrence and metastasis was seen.