Purpose <p>This study aimed to investigate the role of [¹⁸F]fluorodeoxyglucose ([¹⁸F]FDG) positron emission tomography/computed tomography (PET/CT) in survival prediction of interstitial lung diseases (ILD) patients undergoing lung transplantation (LTx).</p> Methods <p>Eighty-three patients with ILD who underwent LTx were retrospectively included in this study, among whom 43 received unilateral LTx and 40 received bilateral LTx. All the patients received [¹⁸F]FDG PET/CT scanning prior to LTx. Clinical characteristics and [¹⁸F]FDG PET/CT parameters were analyzed by using univariate and multivariate analysis to identify predictive factors for overall survival (OS). Correlation of post-operative extracorporeal membrane oxygenation (ECMO) time with clinical and [¹⁸F]FDG PET/CT parameters was analyzed by using Pearson’s correlation analysis.</p> Results <p>Post-operative ECMO time (<i>p</i> &lt; 0.001), age (<i>p</i> = 0.006) and LDH (<i>p</i> = 0.015) were independent predictive factors for OS of unilateral LTx patients. In addition, LDH (<i>p</i> = 0.03) and gender (<i>p</i> = 0.03) were identified as independent factors for OS of bilateral LTx patients. Especially, we found that glucose metabolism (maximum standardized uptake values (SUVmax) of non-transplanted lung, SUVmax_NT) was an independent factor for OS (<i>p</i> = 0.027) of unilateral LTx patients. For patients with bilateral LTx, SUVmin of bilateral lungs exhibited a strong trend in predicting OS (<i>p</i> = 0.08). Furthermore, SUVmean, SUVsum and total lesion glycolysis (TLG) were significantly correlated with post-operative ECMO time.</p> Conclusion <p>This study for the first time suggested that [¹⁸F]FDG PET/CT could be a potential approach in predicting OS of LTx patients with ILD, which might be helpful for the survival evaluation of ILD patients with LTx.</p>