Introduction: Although numerous syndromic and non-syndromic odontogenic lesions of the jaws have been documented in the literature, there are very few cases of simultaneous benign and malignant jaw lesions.
Patient concerns: We present a case of right maxillary squamous cell carcinoma along with several benign odontogenic cystic lesions of the jaws and skeletal abnormalities that meet the criteria for Gorlin-Goltz syndrome.
Takeaway lessons: With a review of the literature, the specifics of management and follow-up are discussed.
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