FH-deficient RCC (also known as HLRCC-associated RCC) is a well-recognised renal cell tumour type (14, 15) which has a distinct histological appearance including characteristic large nuclei with a very prominent organophilic or eosinophilic nucleolus surrounded by a clear halo in addition to FH deficiency on immunohistochemistry. This evidence concerns the gene FH and hereditary leiomyomatosis and renal cell cancer.