Interstitial lung disease (ILD) is a clinical syndrome of various etiologies and histopathologic categorization that, when clinically significant, impair respiratory function. Patients with ILD may develop critical illness from respiratory failure, nonpulmonary organ failure, or after surgical procedures. Additionally, the intensivist must be adept at recognizing exacerbation syndromes, which can complicate the disease course of some forms of ILD. This article discusses mechanical ventilation, noninvasive mechanical ventilation, exacerbation syndromes, and surgical concerns for patients with ILD who are critically ill.