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Introduction Epithelioid trophoblastic tumor (ETT) is a rare neoplasm of the chorionic-type intermediate trophoblast that affects reproductive age women, in most cases with a prior gestation, with vaginal bleeding as beginning presentation. The literature suggests surgery as elective treatment for ETT as it is not responsive to chemotherapeutic agents, used in the treatment of other types of gestational trophoblastic diseases. Patients and methods We report the case of a 31-years-old woman who presented with constipation and abdominal distension (unusual for an ETT). She had an uneventful cesarean delivery three months before. Pelvic ultrasound showed two large adnexal masses (50x60 mm each one), multiple echogenic foci on peritoneum of Douglas space, anterior rectum's wall and small bowel, with a thickening of omentum suggestive of peritoneal carcinosis with omental cake. Endometrial thickness was 2 mm. A huge amount of ascitic fluid reached Morrison space. Beta HCG was mild positive (34mUI/mL) and serum oncomarkers (Alfa1 fetal protein, CEA, Ca 19.9, Ca 15.3, CA125) were negative. Because of the recent pregnancy and positive Beta HCG, a chorionic type disease was thought at beginning, even though symptoms and imaging tend to an ovarian carcinoma. Diagnostic hysteroscopy with multiple endometrial biopsies and pap smear were negative. The diagnostic laparoscopy revealed a diffuse peritoneal carcinosis. Omentum, jejunum, ileum, diaframmatic surfaces, liver's round ligament, surface of the uterus also appeared to be involved and adnexa were transformed in two large masses of 10 cm in diameter. The Fagotti Score was 12; the Peritoneal Cancer Index 28. Morphological features of the tumor and immunohistochemical positivity for CK7, p40, CK 5/6, GATA3, CD10, MUC4, human chorionic gonadotropin (hCG), anti-placental alkaline phosphatase (PLAP) and human placental lactogen (hPL) were consistent with the diagnosis of an extrauterine ETT. Results 6 cycles of neoadjuvant chemotherapy with Etoposide and Cisplatinum, to let feasible the surgical debulking, were performed, but the computed tomography revealed a significant dimensional reduction of the adnexal masses with no differences in the distribution of the peritoneal carcinosis. Despite these findings, we made an explorative laparotomy: the carcinosis process involved the entire pelvis, with nodules of 2-3 cm on the ileum, jejuneum, stomach and liver; she was not operable. Palliative chemotherapy was performed postoperatively and she died from disease after 10 months. Conclusion Extrauterine presentation of ETT, in the absence of uterine lesions, has been rarely reported and it should be considered, although rare, in patients with atypical ovarian/peritoneal cancer or with a gestational trophoblastic tumour, who do not respond to appropriate chemotherapy. The differential diagnosis of ETT includes placental site trophoblastic tumor (PSTT), choriocarcinoma, squamous cell carcinoma, undifferentiated carcinomas and, to make the right diagnosis, an appropriate immunohystochemical panel is essential. Considering the extremely rare incidence of an extrauterine ETT and the limited data published regarding follow-up, we think that our pathological and clinical experience can provide more information on its features and prognosis underlying that diagnosis and management of extrauterine ETT is the result of a multidisciplinary approach with a strong cooperation between gynecologists, pathologists and oncologists. [ABSTRACT FROM AUTHOR]