Primary renal neuroendocrine tumors (NETs) are low-grade tumors with neuroendocrine differentiation and have been reported to arise most commonly in renal abnormalities such as horseshoe kidney. We report a case of a 54-year-old woman with history of invasive ductal breast carcinoma presenting several liver lesions. Histological findings finally revealed liver locations of a well-differentiated NET. Imaging workup combining somatostatin receptor scintigraphy and F-DOPA PET/CT finally enable proper diagnosis of primary renal NET located on the isthmus of a horseshoe kidney.