Germline pathogenic variants in BAP1 are associated with a variety of tumors in the context of the BAP1-tumor predisposition syndrome (TPDS) characterized by familial occurrence of cutaneous melanocytic tumors, uveal melanoma, malignant mesothelioma of the pleura and the peritoneum, renal cell carcinoma and specific non-malignant neoplasms of the skin [2]. Here, BAP1 is linked to renal cell carcinoma.