We analyzed pathogenic germline variants within coding regions of six genes associated with UM tumorigenesis, seven linked to BAP1 functions, and seven confirmed or candidate genes for RCC, a cancer that in our service was the most common nonocular malignancy associated with BAP1‐TPDS, the co‐occurrence of which with UM we at the time also explored in patients outside the present, consecutive series of UM. Here, BAP1 is linked to renal cell carcinoma.