Classification of ameloblastoma into solid or multicystic, unicystic and peripheral types based on the biological behavior has gained recent recognition. Available English-language literature on unicystic ameloblastoma since its first description in 1977 was reviewed. A total number of 182 cases was retrieved to evaluate various clinical and pathological aspects of this variant. The tumor tends to occur at an younger age (mean: 25.5 years) and exhibits a low rate of recurrence (13.8%) following enucleation or curettage in comparison to the conventional ameloblastoma. The variability of histological spectrum, diagnostic problems and choice of treatment for this form of ameloblastoma were also discussed in the light of recent research interest and progress.