Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is a recently described subtype of RCC found in individuals with ACD of the kidney. Because of underrecognition, information regarding this lesion is sparse but continues to accumulate with each new report. Herein, a thorough literature review amassing the current understanding of this unique neoplasm is presented. Discussion focuses on clinical features, pathogenesis, disease outcome, and relation to the duration of dialysis. The macroscopic and characteristic microscopic features are described with illustrations. Compared with previous opinion, compiled immunohistochemical data may now allow for recognition of a unique immunophenotypic pattern of ACD-RCC. Distinction of ACD-RCC from clear cell and papillary RCCs based on molecular genetic information is deliberated, including a summary of the most frequently detected cytogenetic abnormalities. The key morphologic and immunophenotypic patterns used to distinguish this entity from a comprehensive differential diagnosis are provided.