PURPOSE: Interstitial pneumonia (IP) is a progressive and irreversible fibrosis and can be fatal if acute exacerbation (AE) occurs. While a useful risk-scoring system has been established for lung surgery, no risk evaluation exists for AE of IP related to non-pulmonary surgery. The objective of this review is to describe the management for patients with IP.METHODS: We experienced three hepatectomy cases with IP. The first was a 72-year-old male patient diagnosed with hepatocellular carcinoma. Preoperative computed tomography (CT) revealed IP with reticular shadow at the base of both lungs. After hepatectomy, his IP became acutely exacerbated and did not improve with steroid or sivelestat treatment. The second was a 74-year-old male patient diagnosed with hepatocellular carcinoma, and the third was a 75-year-old male patient with liver metastasis. In both these cases, CT revealed a reticular shadow in the lung fields, with increased serum KL-6 levels. We administered pirfenidone for perioperative management, during which time no respiratory complications occurred.RESULTS: Perioperative management with pirfenidone for hepatectomy accompanied by IP was successful in our cases.CONCLUSION: We reviewed reports on the perioperative prevention, intraoperative risk factors, and treatment of postoperative AE of IP and summarized the perioperative management techniques for IP patients undergoing non-pulmonary surgery.