•PNET of the uterus is rare and requires early diagnoses and treatment.•Molecular analysis is important to distinguish it from other tumors.•Different combinations of adjuvant chemotherapy have been report.
Keywords: Carboplatin; EWSR1 gene; Etoposide; Ewing sarcoma; Primitive Uterine neuroectodermal tumor (PNET); Uterine tumors.