Interstitial lung disease (ILD) is associated with a higher incidence of lung cancer, and lung cancer patients with concomitant ILD have a poor prognosis with high mortality and treatment challenges. ILD and lung cancer share similar risk factors and pathogenesis. Treatment of lung cancer may trigger acute exacerbations of ILD or cause ILD as an adverse event. Various agents targeting common pathogenic mechanisms of lung cancer and ILD have been demonstrated to be promising therapeutic drug. This review provides a comprehensive analysis of the epidemiology, risks, and therapeutic strategies of ILD with lung cancer, aiming to enhance awareness and recognition of this disease in clinical practice from an academic perspective.