Background: The aim of this study was to collect, synthesize, and analyze the clinicdemographic data of rhabdomyosarcoma affecting the oral cavity of young children. Methods:The clinical question was "What are the clinical characteristics of oral rhabdomyosarcoma in children aged zero to two years?" Two independent reviewers selected the studies, extracted data, and assessed the methodological quality through the Joanna Briggs Institute Checklist. Descriptive statistics were performed in SPSS.(protocol: osf.io/b9hvy).Results: Thirty case reports with 41 patients were included (22 males; mean age at diagnosis: 15 months). All studies had some methodological limitations, mainly due to the lack of clarity for a final judgment. More than 70% of the cases affected the tongue or lips. Tumors had a fast growth (mean 2.5 months), usually with red coloration, and variable shape and consistency. Embryonal (63.41%) and alveolar (29.27%) were the most common histological subtypes. Treatment was quite variable but usually included surgery. Most patients (53.66%) were alive without disease at follow-up. Conclusion:Oral rhabdomyosarcoma is rare in children aged 0-2 years without a marked gender predilection. The tumor presents as a fast and infiltrative growth leading to local and/or systemic symptoms, and a favorable prognosis for most patients.