Background: Eruption of first primary tooth starts on an average at around six to seven months of age. Presence of teeth at birth or within a month after birth is considered rare. Natal teeth are those present in the oral cavity at the time of birth. These teeth can cause ulcers on the ventral surface of the tongue, lip, and the mother's breast characterizing the Riga Fede Disease. Exact aetiology of natal and neonatal teeth is still unclear. The treatment depends on its mobility of teeth as it is associated with the risk of aspiration or swallowing, whether the natal tooth is supernumerary or primary, causing any problems in breast feeding, presence of soft tissue injuries on tongue of the child or mother's breast and overall health of child. Case presentation: A fifteen day old girl reported with large ulceration on ventral surface of tongue due to sharp natal teeth present at mandibular anterior region which was also associated with feeding difficulties. Along with Riga Fede disease Syndactyly and Oligodactyly in left and right legs respectively was also observed. Extraction of the teeth has been carried out and complete healing of ulceration has been achieved within 30 days. Conclusion: The present case report describes a rare occurrence of Riga Fede disease with Syndactyly and Oligodactyly and highlights its symptomatology and therapeutic approach.
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