ABSTRACT
Neurofibroma is an uncommon benign tumour of neural tissue origin rarely presenting in the mouth and jaws and thereby attracting the attention of oral physicians. A 22-year-old male patient reported with a complaint of swelling in left middle one third region of face since 8-10 y which was slowly progressive in size. He had history of multiple dark brown pigmentation on skin associated with progressively enlarging multiple small nodular growths over the body and single firm nodular growth in left side of maxilla intraorally. He had history of tuberculosis at the age of one year which was treated completely and since last 2-3 y he was suffering from recurrent episodes of sore throat, fever, diarrhea, abdominal pain with vomiting and excessive weight loss. Radiographic findings showed irregular osteolytic lesions involving ramus and angle of mandible, zygomatic bone and posterior part of maxilla with displacement of teeth with abnormal soft tissue enhancement observed by advance imaging. On serological investigation he was HIV positive and histopathologically, diagnosed with Neurofibromatosis-1. Oral manifestations of neurofibromatosis have been reported in only 4% to 7% of affected persons. This article presents a rarest of rare case report of neurofibromatosis-I in HIV positive individual also involving maxilla, mandible as well as zygomatic arch.
