Here, we present the fifth reported case of a 77 year-old male with metastatic HCC presenting as a symptomatic superior sulcus lung tumor and discuss the genomic profile of this rare presentation of HCC for the first time, which included multiple classic mutations in HCC such as TERT, TP53, and WNT/β-catenin signaling as well as in the DNA repair gene ATM. This evidence concerns the gene TERT and hepatocellular carcinoma.