A case of ovarian carcinoma and systemic amyloidosis in a 64 year old woman with nephrotic syndrome is reported. Immunohistochemical study of renal and rectal biopsy specimens revealed the presence of amyloidosis AA antigens, consistent with secondary amyloidosis. The absence of chronic inflammatory or infectious disease suggested the presence of a neoplasm. The gynaecological sonogram showed a solid mass located behind the uterus. Laparotomy confirmed a poorly differentiated ovarian carcinoma. The association of systemic amyloidosis and ovarian carcinoma has not to our knowledge been described previously.