Keratocystic odontogenic tumour (KCOT) is a benign cystic intraosseous tumour of odontogenic origin that is usually solitary except when syndromic. It rarely occurs in the maxilla; therefore a rapidly progressive, nonsyndromic bimaxillary KCOT with locoregional extension poses significant diagnostic and management challenges. To the best of the authors' knowledge, documentation of a nonsyndromic bimaxillary KCOT is nonexistent in the English literature.
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