The discovery of hereditary renal cell carcinoma (RCC) syndromes has had a major impact on modern understanding and management of renal malignancy. Yet, as more familial syndromes and genetic predispositions are described, numerous clinical questions arise. (1) Who should be screened and when? (2) Is a diagnosis of a genetic predisposition to RCC an actionable finding? (3) How does such a diagnosis change immediate and downstream management for a given patient? (4) How should the patient`s family members be counseled regarding the finding?