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Abstract
Tuberous sclerosis is an autosomal dominant disorder that manifests with the formation of hamartomas in multiple organs and systems, including the central nervous system, skin, kidneys, lungs and heart. Patients are affected by seizures status, which are a common feature; also the majority of the patients presents cutaneous findings. Can find intraorally lesions are in tooth enamel constituting the most frequent and rarely present jaw injuries. The Desmoplastic Fibroma is a very rare benign bone tumor, is localized and highly aggressive, is reported in facial bones and other bones of the body and very rarely is associated with tuberous sclerosis. In this article, it presents a case of a 12 year old patient with tuberous sclerosis who presented a lesion in the left maxillary sinus diagnosed as desmoplastic fibroma. Diagnostic methods, clinical presentation and treatment are discussed.
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