Background/Aim: Clear cell carcinoma of the abdominal wall is a sporadic event. To date, about thirty cases have been reported in the literature. This article provides a case report and literature review of an infrequent occurrence with poor prognosis. Case Report: A 45-year-old woman with pelvic pain and an abdominal mass came to our attention. Her medical history was notable for two previous cesarean sections. Physical examination revealed a smooth, multilocular mass measuring about 20 cm, arising from the previous surgical scar. Histology revealed clear-cell carcinoma resulting from the transformation of abdominal wall endometriosis. Given the disease extent, the patient underwent front-line chemotherapy. After several and multiple chemotherapy regimens, there was a disease progression that resulted in the death of the patient in 7 months. The literature review showed that a previous cesarean section was present in 91% of cases. Besides, approximately 26.5% of women died within 12 months of being diagnosed. The mean age of women was 45.88 years, while the average size of the lesion was 11 cm. Conclusion: Clear cell carcinoma is a rare but occurring event. Middleaged women showing an abdominal wall mass in close relation with a surgical scar from a previous cesarean section must be promptly investigated. Treatment options usually include surgery and chemotherapy with poor results. Endometriosis is defined as an inflammatory disease characterized by the presence of a functioning endometrial gland and stroma outside the uterus (1). These lesions usually involve the ovaries and, more rarely, bowel, ureters, lung, and abdominal wall (1). In women with abdominal wall endometriosis (AWE), a prior history of gynecological surgery with the opening of the uterine cavity is usually found. In this regard, scar endometriosis can be explained by iatrogenic transplantation of endometrial tissue to the wound edge during the surgical procedure (2, 3). The incidence of abdominal surgical scar endometriosis ranges between 0.03% and 1.08% of women undergoing pelvic surgery (4, 5). It is often misdiagnosed and detected preoperatively in 20%-50% of cases (2). Women typically report a cyclic menstrual pain referred to the abdominal wall. The differential diagnosis of a subcutaneous mass associated with a previous surgical incision of the abdominal wall includes abscess, hematoma, hernia, desmoid tumor, sarcoma, and metastatic disease (6). An accurate medical history and physical examination can guide towards diagnosis. Definitive treatment includes wide local excision with free margins. Hormonal therapies relieve symptoms but do not prevent recurrences (6). Although endometriosis is considered a benign condition, malignant transformation is rare but possible. About 80% of endometriosis-associated malignancies have been found in the ovary, whereas 20% are localized in extra-gonadal sites (7). Clear cell histology represents 4.5% of the extra-pelvic endometriosis-associated malignancies. It represents the most common histotype in the ...