Pleural mesothelioma (PM) is a rare pleural mesothelial cell malignancy with a poor prognosis. More than 80% PM is associated with prior exposure to asbestos. Asbestos is banned in many countries, but globally the disease burden is expected to rise. Advanced diagnostics and new treatment strategies can improve the prognosis of patients. After more than two decades, a new treatment method – immunotherapy – has been approved for the treatment of inoperable PM.