To evaluate the relationship between mediastinal lymph node enlargement and disease severity score in patients with pulmonary fibrosis. A retrospective study included 30 patients with pulmonary fibrosis: idiopathic pulmonary fibrosis (n = 25), usual interstitial pneumonia (UIP) associated with collagen vascular disease (n = 4), and UIP associated with hepatitis C (n = 1). Disease severity was determined by a computed tomography (CT) scoring system. Each patient's lobe was scored by two radiologists on a scale of 0-5 for both ground glass opacity (GGO) and fibrosis. The presence, number, and sites of enlarged nodes (short axis > or = 10 mm) were assessed. CT severity scores were compared with total number of enlarged lymph nodes (L/Ns) and short axis diameter of the largest L/N (LLN). According to each severity score, patients were divided into two groups: the GGO-predominant group (n = 10) and the fibrosis-predominant group (n = 20). Total numbers of enlarged L/Ns and short axis diameter of LLN were compared in each group. Enlarged mediastinal L/Ns were present in 86%. Total severity score, GGO score, and fibrosis score strongly correlated with total number of enlarged L/Ns (p<0.05). Total severity score and GGO score correlated well with short axis diameter of LLN; however, the fibrosis score did not correlate with the short axis diameter of LLN. In respect to total number of enlarged L/Ns, the difference between the GGO group and fibrosis group was not apparent. In respect to the short axis diameter of LLN, the GGO group LLN was larger in diameter than the fibrosis group LLN (p<0.05). The greater the severity score of pulmonary fibrosis, the larger the total number of enlarged L/Ns. Those patients with more GGO had larger lymph nodes.