Objective: Tracheo-innominate fistula (TIF) is a rare but fatal complication of tracheotomy. To date, there is a paucity of literature regarding pediatric TIFs. The objectives of this study were to conduct a systematic review of literature on pediatric TIF following tracheotomy and describe three demonstrative cases from our institutional experience.Methods: We conducted a systematic review using MEDLINE, Embase, Cochrane Database of Systematic Reviews, Web of Science, and CINAHL. All studies with pediatric patients (under 18 years of age) who developed TIF following tracheotomy were included.Results: Fifty-four publications met inclusion criteria, reporting on 77 cases. The most common indication for tracheotomy was prolonged intubation and the need for ventilatory support (38.6%), with neurological comorbidities being the most common indication (72.7%). The mean time to TIF was 395.7 days (95% confidence interval, 225.9-565.5). Fifty-four patients (70.1%) presented with massive hemorrhage, whereas 18 patients (23.3%) presented with a sentinel bleeding event. The most common diagnostic interventions were computed tomography scan with or without contrast and bronchoscopy (55.8%). A substantial number of patients did not have any investigations (41.6%). Surgical management occurred in 70.1% of patients. Mortality was 38.6% in reported cases with variable follow-up periods.Conclusion: TIF may occur in long-term tracheostomy-dependent children, contrary to the conventionally described 3-week postoperative period. The mortality may not be as high as previously reported with timely intervention. Our results are limited by inherent risks of bias. Further research including well-designed cohort studies are needed to guide an evidencebased approach to TIF.