Primary renal adenocarcinomas comprise of less than 1% of renal and ureteral epithelial tumors. We present a case of a 67-year-old male with a history of simple cystectomy who underwent left nephroureterectomy for primary enteric-type renal adenocarcinoma with cystitis glandularis features. Pathological examination confirmed grade 2 pT1N0MX primary enteric-type renal adenocarcinoma. The patient underwent left open radical nephroureterectomy, with an uneventful postoperative course. Surgical excision is the mainstay treatment, while chemotherapy and radiation are potential adjuncts. Prognosis remains poor, with a 50% overall survival rate within two years of surgery. Further research is needed to enhance treatment recommendations.