Abstract Background and objective IPF is a form of interstitial pneumonia of unknown origin that has a poor prognosis for which current treatments are limited. Recent studies have shown that EMT plays a role in IPF and tumour metastasis. L1 ‐ CAM has also been linked to EMT during tumour development and tumour metastasis. Our aim was to determine prospectively the level of L1 ‐ CAM in IPF patients. Methods Forty consecutive Chinese patients (with IPF , 16; LC , 12; and CC , 12), but no apparent lung or other organ's diseases were enrolled. Soluble L1 ‐ CAM ( sL1 ‐ CAM ), TGF ‐β1, PDGF , γ‐ INF levels in BALF and serum sL1 ‐ CAM were measured using ELISA . Results BALF sL1 ‐ CAM levels of IPF , LC and CC patients were 10.87 ± 0.88 ng/mL, 6.34 ± 0.67 ng/mL and 5.43 ± 0.65 ng/mL, respectively. BALF sL1 ‐ CAM concentration of IPF patients was significantly higher than that in LC and in CC patients. Besides, serum sL1 ‐ CAM levels in patients with IPF , LC and CC were 9.60 ± 1.41 ng/mL, 9.82 ± 0.72 ng/mL and 5.41 ± 1.07 ng/mL, respectively. The serum sL1 ‐ CAM levels in patients with IPF and LC were significantly higher than those in patients with CC ( P < 0.001, respectively). Conclusions The concentrations of sL1 ‐ CAM both in BALF and in serum of patients with IPF are markedly increased compared with controls. This indicates that L1 ‐ CAM might be involved in the pathogenesis of IPF as well as that of LC .