Objective: It is not rare for the radiologist to identify multiple renal masses and be the first to raise the possibility of a hereditary renal tumor syndrome. Characteristic renal and extrarenal imaging findings aid in making the correct diagnosis. The imaging findings, screening guidelines, and management techniques for the most common hereditary renal tumor syndromes are reviewed.
Conclusion: Hereditary renal tumor syndromes have specific screening guidelines and unique management techniques in which imaging plays a central role.