Invasive fungal diseases are severe complication of the lung transplant patients' follow-up as they are increasing the risk of rejection. We report a patient who developed possible Tyromyces fissilis co-infection during graft rejection episode 2 years after bilateral lung transplantation for cystic fibrosis. The fungus was detected using conventional culture methods as a filamentous basidiomycete and further placed to T. fissilis species based on internal transcribed spacer (ITS) rDNA sequences. The patient was treated according to the susceptibility testing results by voriconazole in combination with the anti-rejection therapy and recovered completely within few weeks. This is, to our knowledge, the first published case report of T. fissilis as a possible causative agent of an infection/rejection episode in a lung transplant recipient.