A variety of tumors in the gynecologic tract can be associated with hereditary syndromes. The association between Lynch syndrome and endometrial/ovarian carcinoma and germline BRCA1/2 mutations with pelvic high-grade serous carcinoma is well established. Other well-known syndromes associated with gynecologic neoplasms include Cowden syndrome, Peutz Jeghers syndrome (PJS), and Von Hippel-Lindau syndrome (VHL). The spectrum of gynecologic tumors associated with heritable syndromes has recently grown to include other conditions such as hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC), DICER1 syndrome, and rhabdoid tumor predisposition syndrome (RTPS2). In many of these syndromes, the gynecologic neoplasm is the sentinel malignancy providing an ideal opportunity for the pathologist to aid with early detection of the syndrome which could have important implications for the patients and their family members.