OBJECTIVES:To evaluate the principal features of focal cemento-osseous dysplasia (FocCOD) by systematic review (SR) and to compare their frequencies between four global groups. METHODS:Alternative names for FocCOD were used as search terms. The databases searched were the PubMed interface of Medline and LILACS (Literature Index for Latin-America and the Caribbean; Biblioteca Regional de Medicina (BIREME)). Only those reports of FocCODs which occurred in a series in the reporting authors' caseload were considered. All cases used radiographs and were confirmed fibro-osseous lesions histopathologically. RESULTS:Of the 20 series considered, 10 were included in the SR. Five SR-included series were of East Asian communities. 64% of all SR-included cases were found incidentally. FocCOD predominantly affects females and the mandible. The three predominant radiological presentations varied significantly between reports. CONCLUSIONS:The two at-risk global communities appear to be East Asians and those of black African origin. Although there appears to be little difference between East Asians and non-East Asians, the significant differences between them with regards to the predominant radiological presentation could suggest that either all communities vary in their presentation or that most, if not all, did not reflect the true frequency within their communities. Long-term follow-up of large series that would have revealed the long-term outcomes of FocCODs was lacking. This is necessary because of both FocCOD's predilection of edentulous areas, increasingly required for osseointegrated implants, and its wide differential diagnosis, which includes some lesions normally treated by surgery.