Purpose of review This review examines the current understanding of telomere biology disorders (TBDs) in advanced lung disease, with particular focus on their implications for lung transplantation outcomes and management. Recent findings Recent studies have revealed that TBDs are enriched in lung transplant populations, with many idiopathic pulmonary fibrosis transplant recipients having short telomeres and/or carrying variants in telomere-related genes. While survival outcomes remain debated, recipients with short telomeres consistently show increased susceptibility to cytopenias, cytomegalovirus (CMV) infection, and may require modified immunosuppression regimens. New evidence suggests potential protection against acute cellular rejection in some cases, and novel approaches using letermovir for CMV prophylaxis show promise in managing these complex patients. Summary Management of lung transplant recipients with TBDs requires careful consideration of multiorgan manifestations and individualized management strategies. A multidisciplinary approach incorporating genetics, haematology, and hepatology expertise is increasingly essential for optimal outcomes in this unique population.