Malignant epithelial tumors associated with Recklinghausen disease are very rare. A 32-year-old female with Recklinghausen disease was admitted to our hospital complaining of a 5 X 4 cm left breast mass. A malignant tumor of the breast was diagnosed by mammography, echography and clinical features. Radical mastectomy was performed and pathological examination showed papillotubular adenocarcinoma. Three cases of breast cancer associated with Recklinghausen disease reported in the Japanese literature were reviewed.