Although the profile of malignancies and age at onset among family members partly matched the phenotype of BRCA1- and BRCA2-associated hereditary breast and ovarian cancer, diagnosis of ovarian neoplasm in the proband at an early age, undifferentiated structure of the tumor, and expression of neuroendocrine markers were uncommon for this type of cancer. Here, BRCA2 is linked to ovarian neoplasm.