Endometrial stromal sarcoma (ESS) with primitive neuroectodermal differentiation is a very uncommon entity. Such a case presenting as stage IIIc (International Federation of Gynaecology and Obstetrics (FIGO) 2010) disease in a 51-year-old female is described. Microscopy suggested a small blue round cell tumour. Cytogenetic and multicolour fluorescent in situ hybridisation (M-FISH) analysis revealed a complex karyotype with the presence of unbalanced t(10;17)(q22;p13) translocation, indicating ESS. Peripheral Ewing´s sarcoma was excluded based on FISH and RT-PCR fusion transcripts analysis. After surgical staging, the patient received bleomycin-etoposide-cisplatin combination chemotherapy. A detailed analysis of the histopathology and genetic findings forms the basis of this report.