Purpose of reviewLung transplant (LTx) evaluation and selection of candidates with connective tissue disease (CTD) remains controversial and varies between centers, and the optimal candidate selection is still controversial.Recent findingsRecent United States and European publications have reported reasonable short-term and long-term LTx outcomes in patients with CTD to other lung fibrosis patients without CTD. This article discusses the recently published International Society for Heart and Lung Transplantation (ISHLT) consensus document recommendations to evaluate and select CTD candidates, the importance of early referral, posttransplant management, and the involvement of a multidisciplinary team.SummaryFuture standardized practices among centers adapting the 2021 ISHLT consensus recommendations to evaluate and select CTD candidates will allow risk stratification, determine the best candidates, and facilitate the most successful long-term LTx outcomes.