Adenomatoid odontogenic tumour (AOT) is a benign tumour that arises from odontogenic epithelial remnants. AOTs usually present as innocuous lesions with limited growth potential. Multiple AOTs are frequently reported in the literature, with reports of tumours associated with up to seven impacted teeth. Multiple AOTs have also been described in association with Schimmelpenning syndrome. This case report highlights the rare occurrence of multiple AOTs involving eight impacted teeth in a 9-year-old male patient. Radiographic examination showed features of enamel hypoplasia and multiple prominent dilated gubernaculum dentis associated with some of the impacted teeth. The patient also presented with a linear epidermal nevus involving the left face and intraoral mucosal papillomatous growths, clinical features highly compatible with Schimmelpenning syndrome.
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