This study was conducted in order to assess the role of MR imaging characteristics of HLRCC-associated cystic lesions for distinguishing benign from malignant complex renal cysts in this patient population.<h4>Methods</h4>This IRB-approved retrospective study included 42 HLRCC patients (mean age, 46 ± 14 years; men: women, 22:20) with a pathogenic FH germline variant with renal cysts on abdominal MRI. This evidence concerns the gene FH and Renal cyst.