Von Recklinghausen\u27s disease is an autosomal dominant characteristic syndrome including cutaneous pigmentation, neurofibromatosis, soft tissue hypertrophy and abnormalities of skeleton. The present report is a case of von Recklinghausen\u27s disease, observed in a 53-year-old man, which is accompanied by multiple neurofibromas, pigmentation of skin and mild scoliosis. On examination, neurofibroma was located in the midline of the dorsum just anterior to the circumvallate papillae of the tongue. 2.0 cm vertical incision over the tumor through the mucosa and blunt dissection exposed the entire tumor. It was easily freed from the surrounding tissue and enucleated. Healing proceeded well without evidence of recurrence. We discussed the case with reference to literature concerning oral lesions over the last twenty years
