Background: Endometrial giant cell carcinoma (EGCC) is a rare form of endometrial carcinoma (EC) with limited documentation in medical literature, consisting of only two small series and a few case reports. Till now, the World Health Organization (WHO) classification of endometrial neoplasms does not recognize EGCC as a distinct entity. Due to its rarity, there is a lack of comprehensive data on the biological characteristics, clinical management and prognosis of EGCC. Cases: In this report, we present a rare case of EGCC in a 55-year-old postmenopausal woman, describe its clinicopathologic features and review the relevant literature. The patient underwent a comprehensive surgical procedure, including a total abdominal hysterectomy, bilateral salpingo-oophorectomy and pelvic lymphadenectomy. Postoperative radiochemotherapy was not administered, and no evidence of disease recurrence was observed during the one-year follow-up period. Conclusions: Although there is insufficient evidence to definitively classify EGCC as a distinct uterine malignancy, our review of the relevant literature suggests that it may exhibit aggressive behavior based on its clinicopathological and molecular characteristics, and the presence and/or percentage of giant cell components should be clearly mentioned in pathological reports to highlight uncertainty regarding its biological characteristics. Further accumulation of experiences is necessary to improve the diagnosis and treatment of this uncommon tumor.