Intraoral desmoplastic fibroma: a manifestation of tuberous sclerosis

Desmoplastic fibroma (DF) is a benign but locally aggressive tumor usually recognized as an intraosseous counterpart of soft tissue fibromatosis in both gnathic and extra-gnathic sites. The most common site is mandible followed by pelvis and long bones. Maxilla has been reported to be involved very rarely. Desmoplastic fibroma is recently being considered to be one of the oral manifestations of tuberous sclerosis rather than a coincidental finding. We report an unusual case of a girl with a previous diagnosis of tuberosclerosis who presented at 8 years with an oral lesion diagnosed as desmoplastic fibroma.