Primary neuroendocrine tumor of kidney is extremely rare .We present a case of the same originating in the horseshoe kidney in a 38-year-old lady with concomitant ovarian metastases. Though the patient underwent resection with curative intent followed by adjuvant chemotherapy, she developed brain metastases within one year. Though literature suggests a rather indolent course of the disease, this patient expired of rapidly progressive disease. The report highlights the fact that a very high index of suspicion for a neuroendocrine tumor needs to be aroused particularly when confronted with a tumor in a horseshoe kidney moiety.