AbstractHepatocellular carcinoma (HCC) is a common cancer and is frequently diagnosed at a late and unresectable stage with limited effective treatment options. 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 asTERT,TP53, andWNT/β‐catenin signaling as well as in the DNA repair geneATM. The patient was treated with palliative radiotherapy to the Pancoast tumor followed by atezolizumab plus bevacizumab and passed away 6 months after diagnosis. This rare case highlights the need for effective treatment in aggressive and unresectable HCC and the utility of early genomic studies to allow for targeted therapy such as poly (ADP‐ribose) polymerase (PARP)‐inhibitors.