Abstract
INTRODUCTION: Neurofibromatosis type 1 or Von Recklinghausen disease is a rare autosomal dominant hereditary disease with total penetrance. It is characterized by an extreme clinical variability that is also found within the same family. PRESENTATION OF CASE: We report a case of neurofibroma exceptional by its location in the breast in a 40-year-old woman with Von Recklinghausen disease, discovered by a breast nodule on self-examination. A biopsy-exeresis had concluded on the anatomopathological examination to the diagnosis of neurofibroma. DISCUSSION: Breast involvement in neurofibromatosis is extremely rare and possible. Rapid and adequate management of patients with Von Recklinghausen disease is essential in order to make the diagnosis early and to institute appropriate treatment as soon as possible, given the risk of possible malignant transformation. CONCLUSION: Diagnosis is essentially based on anatomopathological study. This confirms the diagnosis and rules out possible associated malignancy.