Background: Patients with nonmalignant respiratory diseases have limited access to palliative care services and health professionals do not adequately address discussions about end-of-life care preferences. Objective: The aim of this systematic literature review was to highlight key components and challenges for patients and health professionals discussing end-of-life care in nonmalignant respiratory disease. Design: A mixed methods systematic review was conducted. Included studies were assessed for quality and data were synthesized thematically, while original data were presented in tabular form. Data Sources: PubMed, CINAHL, BNI, ASSIA, PsycINFO, Science Direct, and Web of Science were searched (1999-2010) for studies on end-of-life discussions. Additional studies were identified by hand searching key journals and reference lists of included articles. Results: Fourteen studies were identified. Three themes involving components and challenges in end-of-life discussions were identified: the discussion, the health professional/patient relationship, and patient perceptions. Conclusions: End-of-life discussions should be initiated by health professionals, who must be aware of patient expectations regarding palliative care and end-of-life care planning. Efforts must be made to develop relationships with patients with terminal illness and allow sufficient time to discuss the end of life during clinical encounters. Future research should address palliative care uptake in nonmalignant disease and implications for health education should be addressed.