In this paper, we report a case of primary squamous cell carcinoma of the endometrium (PSCCE) with a literature review. A 64-year-old woman was admitted because of postmenopausal bleeding. The gynecological exam found bleeding from the endocervix. The pelvic ultrasound objectified uterine regular contours, endometrial thickened was 10 mm, the presence of an intra cavitary lesion measuring 56/70 mm. The diagnostic hysteroscopy revealed a whitish appearance taking all the uterine cavity making evoke a tumor of the endometrium. Pelvic MRI showed a tumor limited to the uterine corpus endometrium (invasion by more than 50% of the myometrium) without invasion of the cervix. Radical hysterectomy, bilateral salpingo-oophorectomy, and lymph nodes dissection were performed. Grossly, the endometrial carcinoma was polypoid tumor occupying the entire uterine cavity. Histologically, the diagnosis of SCC was retained. No adenocarcinoma element was recognized. Neither squamous metaplasia nor dysplasia was recognized. No ectopic cervical tissue was found. The SCC was found to invade into deeper one half of the myometrium. No tumor cells were seen in other sites including the cervix, ovaries, parameters, and lymph nodes. The patients was FIGO 2009 stage IB (pT1B, N0), and was treated with adjuvant radiation. The patient had a disease progression in the pelvis 3 months after the irradiation. We reported a case of PSCCE which can help to enrich the literature for the treatment and prognosis of this disease.