Search criteria were applied to PubMed, EMBASE and the Cochrane Review databases and included all studies published up to January 2023 reporting 3D 4K exoscope-assisted surgeries in pediatric patients. After the removal of duplicates, selection of abstracts and full-text articles, and quality assessment, we reviewed eligible articles for number of patients treated, age, surgical procedures, and outcomes. Among 54 potentially relevant records, 5 studies were considered eligible and included in this systematic review, with reported treatment data for 182 patients. The surgical procedures belong to the otologic field (121 cases), head and neck surgery (25 cases) and transoral surgery (36 cases). Exoscopy allowed high quality visualization of anatomical structures during cochlear implantation and during reconstruction in head and neck surgery; moreover, it improved the surgical view of surgeons, spectators and ENT students. The use of 3D 4K exoscopy has shown promising potential as a valuable tool in pediatric ORL-head and neck surgery; nevertheless, further validation of these encouraging outcomes is necessary through larger-scale studies specifically focused on pediatric patients.