A 50-year-old Asian Indian woman, who had been menopausal for two years, presented with complaints of post-menopausal bleeding for three to four months. A per speculum examination of our patient showed a 10×8 cm vascular congested mass abutting the introitus. A polypoidal mass with a thick pedicle was felt coming out of her endocervix on per vaginum examination. Cervical polypectomy was done on our patient and a histopathological diagnosis of small round cell tumor was arrived at. Following polypectomy our patient continued to have vaginal bleeding. Ultrasonography (USG) and magnetic resonance imaging (MRI) of the whole abdomen and pelvis of our patient revealed an irregular infiltrative mass lesion in her uterine corpus and cervix. No parametrial invasion or significant lymphadenopathy, however, was noted. Subsequently, a total abdominal hysterectomy with bilateral salpingo-oophorectomy with pelvic omentectomy and pelvic lymph node sampling was done on our patient. Her uterus and the cervix grossly measured 8×4×3 cm. On cut surface a polypoidal, soft, tan tumor, was seen filling her entire endometrial cavity and extending to her endocervix. Histology revealed a tumor with majority of the areas displaying primitive neuroectodermal differentiation and a minor component of adenosarcoma constituting around 20% of the tumor volume. The former was composed of sheets of undifferentiated, small round to oval cells with hyperchromatic nuclei and numerous mitotic figures. Micronodular pale islands with fibrillary matrix, perivascular pseudorosettes and occasional Homer-Wright rosettes were seen. Areas of adenosarcoma comprised of epithelial lined cleft-like spaces and glands showing mild atypia and occasional mitosis along with sarcomatous stroma and polypoidal stromal projections into the lumen. These glandular structures were present well away from the residual endometrium and were not accompanied by the normal endometrial stroma. Lymphovascular emboli were also seen. The tumor showed full thickness myometrial invasion and extension into the cervix. Left parametrium also showed tumor infiltration and one left external iliac lymph node showed metastasis. On immunohistochemistry (IHC), both spindle cell component of the adenosarcoma and small cell component were positive for vimentin. The small cell component also showed strong positivity for neuronspecific enolase (NSE), and synaptophysin. Chromogranin and cytokeratin examination results were negative. CD99 was also negative. A final diagnosis of stage IIIC PNET with adenosarcoma of the uterus was finally made. Two months post-operatively, our patient again presented with bleeding per vaginum and a nodule at the vaginal vault. Her whole abdomen computed tomography (CT) scan showed normal upper abdomen with a 2×2-cm mass at the vaginal vault. She was then started on radiotherapy and chemotherapy. Until her last follow-up examination at six months after her surgery, our patient has received a total of three cycles of radiotherapy and six cycles of chemotherapy. Her vault nodule has been completely regressed and she is now disease-free.