Clear cell papillary renal cell carcinoma is a relatively recently recognised variant of renal cell carcinoma and has identical features to other tumours variously classified as clear cell papillary and cystic renal cell carcinoma, clear cell tubulopapillary renal cell carcinoma, renal angiomyoadenomatous tumour, and sporadic renal cell carcinoma with diffuse CK7 positivity [3, 5, 10]. The gene discussed is KRT7; the disease is hereditary clear cell renal cell carcinoma.