Introduction: Small cell neuroendocrine differentiation (NE) in malignant mixed Mullerian tumors (MMMTs) is a rare and unusual occurrence with very few previously reported cases. There is no consensus regarding its diagnosis, classification, and optimal treatment options. Case: The authors report a patient with endometrial MMMT and NE differentiation who initially received comprehensive surgery followed by adjuvant chemotherapy containing cisplatin and etoposide. She further underwent metastasectomy and received carboplatin and paclitaxel for the relapse. She is still alive 12 months after the diagnosis. The authors performed a review of literature in order to characterize the clinical phenotype. These patients have a very aggressive disease. Median life expectancy seems to be less than a year. Conclusions: It is reasonable to perform comprehensive staging surgery followed by adjuvant chemotherapy irrespective to stage of the disease.