Advanced restrictive lung diseases remain a challenge for both the clinician and patient alike. Because there are few available treatment options that prolong survival for patients with diseases such as idiopathic pulmonary fibrosis, improvement in quality of life and palliation of significant symptoms become realistic treatment goals. Several validated instruments that assess quality of life and health-related quality of life have demonstrated the dramatic impact that lung disease has on patients. Quality-of-life assessments of patients with interstitial lung disease have commonly cited respiratory complaints as problematic, but other distressing symptoms often not addressed include fear, social isolation, anxiety, and depression. Not only do respiratory symptoms limit this patient population, but the awareness of decreased independence and ability for social participation also has an impact on the quality of life. Some patients describe a deepened spiritual well-being during their disease process; however, many patients' mental health suffers with experiences of fear, worry, anxiety, and panic. Many patients express desire for more attention to end-of-life issues from their physicians. Fears of worsening symptoms and suffocation exist with an expressed desire by most to die peacefully with symptom control. Interventions to improve quality of life are largely directed at symptom control. Pharmacologic and nonpharmacologic interventions have been helpful in relieving dyspnea. Studies have demonstrated that the use of supplemental oxygen in the face of advancing hypoxemia can have both positive and negative effects on quality of life. Patients using nasal prongs describe feelings of self-consciousness, embarrassment, and social withdrawal. Pulmonary rehabilitation is recommended, with some studies noting increased quality-of-life scores and decreased sensations of dyspnea. Sleep deprivation and poor sleep quality also have a negative impact on quality of life. Recognition and correction of nocturnal hypoxemia and other sleep disturbances should enhance quality of life in patients with restrictive lung disease; however, there is currently no evidence to support this claim. End-of-life care needs more attention by clinicians in the decision-making and preparatory phase. Physicians need to maintain their focus on quality-of-life issues as medical management shifts from curative therapies to comfort management therapies. Palliative care and hospice appear to be underused in patients with advanced diseases other than cancer. Because the only curative option for some end-stage restrictive lung diseases is lung transplantation, if transplantation is not an option, palliation of symptoms and hospice care may offer patients and families the opportunity to die with dignity and comfort.