HighlightsOnly case in literature without history of endometriosis, on Hormone replacement therapy.Only the second case with isolated omental lesion.Extrauterine Endometrial Stromal Sarcoma (EESS) is an extremely rare mesenchymal tumour.This condition can simulate chronic or acute abdominal pathologies.The tumour can occur without preceding endometriosis, and in upper abdominal location.Biopsy showing typical immunohistochemistry markers is the best way to achieve diagnosis.Hormone replacement therapy may be an independent risk factor for EESS occurrence.