Endometrial small cell carcinoma (ESCC) is an extremely rare and aggressive tumor with poor prognosis. It is characterized by early regional and systemic spread leading to rapid development of lymph nodes, pelvic and extrapelvic metastasis and compromising the outcome. In this paper, we reported three cases of ESCC confirmed by pathological and immunohistochemistry studies. In one case, ESCC was associated with endometrioid carcinoma and carcinosarcoma, while the other two cases were pure ESCC. Two cases were diagnosed at early stage IA of the International Federation of Gynecology and Obstetrics (FIGO) cancer staging system. They were treated by surgery followed by pelvic external radiation and brachytherapy with favorable outcome (no recurrence was confirmed and a survival was 1 and 5years, respectively). The third case was diagnosed with visceral metastasis and was treated with 6 cycles of cisplatin plus etoposide. She died 8months after diagnosis. Due to its rarity, there is no standard guideline for the management of ESCC. Its treatment is extrapolated from that of both, the conventional endometrial carcinoma and the small cell carcinoma of the lungs, which share similarities with ESCC. Thus, multimodal therapeutic including surgery, radiation therapy and chemotherapy, seems to be the best therapeutic approach. Randomized clinical trials with multiples cases of ESCC are encouraged to clearly define the optimal therapeutic approach to this rare tumor.