Although TERT promoter mutations have been described as an early event in hepatocellular carcinoma on cirrhosis, they were not observed in an exome sequencing analysis of 18 classical hepatoblastoma specimens, but appeared to be an exclusive feature of transitional liver cell tumors with clinical and histo-pathological features reminiscent of hepatocellular carcinoma, thereby providing an excellent marker for the detection of high-risk patients [171,203,204]. The gene discussed is TERT; the disease is hepatocellular carcinoma.