The design and provision of quality pediatric palliative care should prioritize issues that matter to children and their families for optimal outcomes. This review aims to identify symptoms, concerns and outcomes that matter to children and young people ("young people") with terminal illnesses and their families. Findings from the systematic review will informthe development of a relevant framework of health outcomes. This is a systematic literature review across multiple databases for identification of eligible primary evidence. Data sources includedPsycINFO, MEDLINE, Embase, CINAHL, OpenGrey, and Science Direct Journals, searched from 1 August 2016 to 30 July 2017. The study also incorporates consultations with experts in the field, citation searches via Scopus, and a hand search of reference lists of included studies. Of the 13,567 articles that were evaluated, 81 studies were included. Most of these studies (n=68)were from high-income countries and foused on young peoplewith cancer(n=58). A total of 3236 young people, 2103 family carers, 108 families, and 901 healthcare providers were included in the studies. Young people did not contribute to data in 30% of studies. Themes on priority concerns are presented by the followingdomains and health outcomes: (1) physical (n = 62 studies), e.g., physical symptoms; (2) psychological (n = 65), e.g., worry; (3) psychosocial (n = 31), e.g., relationships; (4) existential (n = 37), e.g., existential loss; and (5) "other" (n = 39), e.g., information access. Burdensome symptoms and concerns affect young people with malignant and nonmalignant conditions and occur across the disease trajectory; pediatric palliative care should not be limited to the end-of-life phase. A child-family-centered framework of health outcomes, spanning the patient, family, and quality of service levels is proposed to inform service development. Future research should address gaps identifiedacross the literature (i.e.,the involvement of young people in research, evidence for developing countries, and a focus onnonmalignant conditions.