Solitary Median Maxillary Central Incisor (SMMCI) is a developmental anomaly in the permanent dentition with one single central incisor in the maxilla, positioned exactly in the midline. This condition has been associated with extra- and intraoral malformations in the frontonasal segment of the cranium and face. It is not known whether the centrally located permanent incisor is always preceded by a centrally located primary incisor. The aim was to analyse whether a permanent single central incisor in SMMCI is always preceded by a primary single central incisor and to study extra- and intraoral phenotypic traits of the condition.