Background: Children with tracheostomy are frequently admitted to the hospital for tracheostomy-associated respiratory infections (TRAINs). However, there remains a paucity of evidence to direct the diagnosis, treatment, and prevention of TRAINs. An important first step to addressing this knowledge gap is to synthesize existing data regarding TRAINs to inform current practice and facilitate innovation. Data Sources: We searched PubMed, Embase, Cochrane Library, CINAHL, and Web of Science from inception to October 2020. Original research articles and published abstracts including children and young adults 0-21 years of age with tracheostomy were included. Included studies assessed the clinical definitions of and risk factors for TRAINs, microbiologic epidemiology and colonization of tracheostomies, and treatment and outcomes of TRAINs. Data Synthesis: Out of 5755 studies identified in the search, 78 full-text studies were included in the final review. A substantial number of studies focused on the detection of specific pathogens in respiratory cultures including Pseudomonas aeruginosa. Several different definitions of TRAIN including clinical, microbiologic, and laboratory testing results were utilized; however, no uniform set of criteria were identified. The few studies focused on treatment and prevention of TRAIN emphasized the role of empiric antimicrobial therapy and the use of inhaled antibiotics. Conclusions: Despite a growing number of research articles studying TRAINs, there is a paucity of prospective interventional trials to guide the diagnosis, treatment, and prevention of respiratory disease in this vulnerable population. Future research should include studies of interventions designed to improve short-and long-term respiratory-related outcomes of children with tracheostomy.