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Background: Midline diastema in children is a prevalent developmental entity, and this pathological condition may remain in many children due to various factors. Nonetheless, the evidence on etiological factors of the midline diastema in children is minimal. Purpose: To evaluate the etiological factors of midline diastema causes in children below 12 years of age from the published data. Methods: A literature search was confined to the English language using MeSH terms conferring to PICO format in PubMed, Cochrane Library, and Ovidsp covering the period from January 1960 to December 2019. Search in Google Scholar, grey literature, and hand search on references were performed to find additional data. Suitable studies were selected based on the predefined inclusion and exclusion criteria. Quality analysis of the chosen studies conducted using the Newcastle-Ottawa Scale (NOS) adapted for cross-sectional studies. Results: Only eight studies were available for final analysis among those four studies from India, two studies from Korea, one study from Brazil, and another study from Canada. The most common etiology for midline diastema was supernumerary teeth followed by morphology labial frenum and nasal airflow condensation. The quality analysis of these studies based on NOS showed one study with unsatisfactory, four studies with satisfactory, and three with good quality. Conclusion: Morphology of frenum, pre-maxillary supernumerary teeth, and nasal airflow condensation seem to be the most common causes of midline diastema in children below 12 years. [ABSTRACT FROM AUTHOR]