Germline mutations in BAP1 result in an autosomal dominant tumour predisposition syndrome that is associated with a high risk of developing various tumours, including BIMT, cutaneous melanoma, uveal melanoma, mesothelioma, renal cell carcinoma, lung adenocarcinoma and meningioma.2 The gene discussed is BAP1; the disease is hereditary clear cell renal cell carcinoma.