In our study, the number of cytotoxic T cells in BHD-associated kidney cancer is quite small and HIF-VEGF signaling is not upregulated in BHD-associated vasculature, suggesting that neither immune checkpoint inhibitors nor angiogenesis inhibitors may be effective against this cancer; therefore, novel therapeutic approaches are needed based on the molecular context of BHD-associated kidney cancer. Here, VEGFA is linked to kidney cancer.