Summary Fibrolamellar carcinoma is a highly aggressive liver cancer that is increasing in frequency world-wide and occurs primarily in children to young adults with no history of chronic liver disease. Recently it was reported that a novel fusion gene, DNAJB1-PRKACA, serves as a marker of this devastating disease. It has not yet been determined whether the chimeric protein encoded by the fusion is the key oncogenic driver. Further studies are needed to determine whether the chimera is an effective therapeutic target and also whether it is secreted into the circulation, which would enable early and non-invasive diagnosis, increasing the likelihood of successful surgical intervention. This article is protected by copyright. All rights reserved.