The purpose of this study was to review the clinical, radiological, and histopathological profile of unicystic ameloblastoma (UA) in children using data from available case reports and reviews published between 1992 and 2012. A total of 513 publications were evaluated. A structured search of the literature was performed, with predefined criteria, using computer and manual searches. An evaluation and critical appraisal was done in three separate rounds. A total of 25 articles describing 51 cases satisfied the selection criteria and were thus included in the final review. UA was mostly found to be located in the mandible, often exhibiting a unilocular radiographic image. Enucleation alone resulted in the highest recurrence rate. According to the results of this study, luminal UAs are less aggressive and respond better to conservative treatment. In contrast, plexiform and mural types frequently result in recurrence. The use of Carnoy's solution in preventing recurrence remains to be established.