Abstract Introduction/Objective Well differentiated neuroendocrine tumors (WDNETs) of the kidney are extremely rare and are aggressive tumors with higher rate of metastasis, even those with low proliferative rates. Primary WDNET arising in a renal teratoma is an exceedingly rare entity. Methods/Case Report Herein, we describe a case with a primary WDNET and possible mature cystic teratoma of the right kidney of a 22-year old male who presented with an epigastric/right upper quadrant abdominal pain. Abdominal MRI identified a 4.4cm isointense mass in the mid-lower pole of the right kidney within renal sinus. Robotic assisted partial nephrectomy was performed. Grossly, the 56gm excised mass is well circumscribed with tan- brown friable homogeneous cut surface. Histologically, tumor cells show round to oval nuclei with speckled chromatin and calcifications. The tumor metastasized to two hilar lymph nodes. The tumor is positive for AE1/AE3, vimentin, synaptophysin, CD99, INI-1 (SMARCB1), FLI-1, and chromogranin. While negative for CK7, CK20, PAX-8, CD10, WT-1, CEA, and CDX-2. Ki-67 is 2% and the mitotic count is < 1 per 10 HPF. The histology and immunoprofile are consistent with WDNET. No pathogenic fusions detected on Next Generation Sequencing (NGS) targeting RNA and DNA panels. Adjacent to the tumor, there is a cystic structure lined by intestinal mucosa positive for CDX2. Results (if a Case Study enter NA) N/A Conclusion Neuroendocrine cells are not identified in the normal parenchyma of the kidney, while noted in adjacent intestinal mucosa raising the possibility that renal WDNET is arising from mature renal teratoma.