Patients with end-stage renal disease and acquired cystic kidney disease are at increased risk of development of renal tumors, and the histologic spectrum of tumors in this setting differs from that of the general population. Two histologies enriched in this context include acquired cystic kidney disease–associated renal cell carcinoma and clear cell papillary renal cell carcinoma, both now considered distinct tumor entities in the International Society of Urological Pathology Vancouver Classification of Renal Neoplasia and 2016 World Health Organization Classification. Acquired cystic kidney disease–associated renal cell carcinoma prototypically demonstrates cribriform architecture, eosinophilic cells, prominent nucleoli, and intratumoral calcium oxalate crystals. However, some characteristics overlap with papillary renal cell carcinoma, including diffuse labeling for α-methylacyl-CoA racemase. Cysts in acquired cystic kidney disease (“atypical” cysts) are thought to represent its precursor lesion, often sharing similar cytology and architecture. Clear cell papillary renal cell carcinoma, in contrast, may closely mimic clear cell renal cell carcinoma; however, its behavior is highly favorable, with no convincing examples of metastases to date. This diagnosis can be supported by immunohistochemical positivity for cytokeratin 7, carbonic anhydrase IX, GATA3, and high-molecular-weight keratin, with lack of reactivity for AMACR and CD10. Despite enrichment for these tumor types, a wide variety of renal tumors also occur in end-stage renal disease, including more well-known histologies, such as clear cell and papillary renal cell carcinomas, as well as papillary adenoma and a newly recognized subtype of vascular tumor, anastomosing hemangioma.