The present report follows the case of a young boy with solitary median maxillary central incisor (SMMCI) syndrome between the ages of 4 and 7 years. This condition is characterized by the presence of one single maxillary central incisor in the midline instead of two central incisors. No other developmental abnormalities involving growth or brain function were noted at, or subsequent, to birth. This report includes a discussion of the aetiology of SMMCI syndrome and its association with birth defects such as holoprosencephaly (HPE), CHARGE and VACTERL, as well as a discussion of the long-term prognosis and associated dental and medical issues for this particular patient
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