Metaplastic carcinomas of the breast are uncommon lesions and account for < 5% of all breast malignancies. A type of metaplastic carcinoma is carcinosarcoma, an even more rare lesion. We present a case of carcinosarcoma in association with Von Recklinghausen's disease in a 60-year-old woman. Von Recklinghausen's disease is an autosomal dominant disorder that is localized at the long arm of chromosome 17 and affects 1 in 4000 individuals. There are few reports on breast tumor development and incidence in patients with Von Recklinghausen's disease. To our knowledge, there has been no report of breast carcinosarcoma associated with Von Recklinghausen's disease. We comment on the difficulties of diagnosis and treatment and the possibility of breast tumors occurring more frequently in patients with Von Recklinghausen's disease.