Neurofibromatosis Type 1 (NF 1) although not a very uncommon tumor presents difficulties in understanding because of lack of in depth knowledge about the etiopathogenesis of the disease at a molecular level, which mainly encompasses knowledge regarding the NF 1 Gene and the protein Neurofibromin.The disease affects several organs in the body and produces a plethora of clinical manifestations. A good percentage of cases also transform into the Malignant peripheral nerve sheath tumors (MPNSTs)thus establishing this entity as a premalignant condition. Hence, this calls for an early and accurate diagnosis in order to decrease the morbidity of the patient.However, this may not be always easy because a good number of other conditions frequently overlap this disease entity.The oral manifestations of this disease seldom receive importance. Thus the aim of the present study was to focus on the etiopathogenesis of the disease along with the oral manifestations.