We report a very rare case of endometrioid adenocarcinoma arising from abdominal wall endometriosis in the appendectomy scar. A 47-year-old woman visited the surgical department, since she had a gradually growing and painful tumor both in an appendectomy scar and at an umbilical site. She underwent appendectomy at age 18 years, and noticed the tumor at age 22 years. Partial tumor resection was performed in that department, and the pathology revealed endometrioid adenocarcinoma. She was referred to our department for radical therapy. Tumors in the both sites were dissected together with some swelling lymph nodes in our department. A pathological diagnosis of the tumor in the umbilical site showed only benign endometriosis. In contrast, the tumor in the appendectomy scar showed benign endometriosis, atypical endometriosis and well differentiated endometrioid adenocarcinoma. Resected lymph nodes also contained endometrioid adenocarcinoma, and were diagnosed as metastases. It was concluded that the endometrioid adenocarcinoma in the tumor of the appendectomy scar was a malignant transformation arising from abdominal wall endometriosis from the pathological findings. Since the operation, adjuvant and maintenance chemotherapy with paclitaxel and carboplatin had been administered for 3 years. She is free of disease 3.5 years after the operation.