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Introduction: Histologically, endometrial neuroendocrine carcinoma is an extremely rare pathological type of endometrial cancer. In addition, this type is characterized by high invasiveness and poor clinical outcome, which was classified into carcinoid (low grade), and small-cell and large-cell neuroendocrine carcinoma (high grade). Globally, reports on endometrial carcinoid carcinoma are limited. Clinically, it is also rare to see primary squamous cell carcinoma of endometrium. Case Presentation: Here, we report an interesting case of mixed carcinoma of endometrium with both carcinoid and squamous cell carcinoma, which presented with persistent vaginal bleeding and hyponatremia. Conclusion: Careful pathologic review is necessary to diagnose this rare disease. More studies in the future are warranted to demonstrate the primary surgical treatments and the efficacy of adjuvant therapy of this disease.