“…The presence of a solid fatty renal lesion, eg, angiomyolipoma, can point toward tuberous sclerosis; this diagnosis is supported by characteristic dermatologic findings, chest computed tomography (CT) with lymphangioleiomyomatosis, and brain MRI to identify subependymal tubers . A diagnosis of VHL disease is suggested by retinal and central nervous system hemangioblastomas, endolymphatic sac tumors, renal cysts, renal cell carcinoma (RCC; clear cell type), pancreatic cysts and tumors, pheochromocytoma, and epididymal cystadenomas . Gene testing, together with genetic counseling and imaging, are often required to confirm the diagnosis of cystic kidney diseases …”