Desmoplastic fibroma of the maxilla in a 5-year-old patient with Tuberous Sclerosis Complex (TSC): Case report and review of the literature

The following case report details the presentation of a left maxillary painless expansile lesion in a five-year-old female that was proven to be desmoplastic fibroma (DF) of the maxilla, which was treated via a conservative excision. Given the paucity of DF cases in the Maxillofacial literature, there are no formally agreed-upon guidelines for the treatment of DF, especially in the maxillary sinus. A thorough review of the literature was completed and discussed, highlighting the correlation of DF with Tuberous Sclerosis Complex (TSC) and the proposed treatment when encountered in the maxillary sinus of a pediatric patient. •Many surgical options have been suggested for maxillofacial DF, without agreed upon consensus.•Maxillofacial DFs are rare tumors with fewer than 160 reported in the English literature.•Although benign, this tumor can grow rapidly and be destructive, complicating treatment.•Maxillofacial DFs may be a manifestation of Tuberous Sclerosis Complex (TSC).•In this case we propose a conservative excision of DF in the maxillary sinus.