Crizotinib is an efficacious and well-tolerated drug in the management of ALK-positive lung cancer. Crizotinib treatment, however, is rarely complicated by the occurrence of acute interstitial lung disease (ILD) that is often fatal. There is no treatment for this serious adverse event. We report a female non-small cell lung cancer patient who developed ILD after a few days of crizotinib therapy. She showed a significant improvement after a high dose of pulse corticosteroid therapy, both radiologically and clinically. Unfortunately, the patient subsequently developed an aspergillosis superinfection leading to death. Our experience suggests that high-dose steroid therapy may be efficacious in the management of a severe complication of crizotinib therapy. However, potent antifungal therapy should be considered to prevent the risk of severe aspergillosis.