INTRODUCTION: Endometriosis affects 3 to 10 percent of women of reproductive age. Most of the time it involves the pelvis; however, sites of endometriosis have been reported almost anywhere in the body. Appendiceal and primary umbilical endometriosis are considered rare loci, making accurate diagnosis elusive. Here we present the case of a 46-year-old woman with concurrent appendiceal and umbilical endometriosis.CASE PRESENTATION: A 46-year-old Greek woman presented with a large mass in the lower abdomen adhering to the surrounding organs. She reported recurrent lower abdominal and pelvic pain and the presence of a dark-blue hard nodule at the umbilicus. She had no previous medical, surgical or gynecological history. Her physical examination and laboratory test results were without any significant findings. The laparotomy revealed a fibromatose uterus adhering to the rectum and a urinary cyst and a palpable mass in the vermiform appendix. A hysterectomy and an appendectomy were performed. The umbilical mass was also excised. Pathology revealed endometriosis of the umbilicus and the appendix. The postoperative period was uneventful and she was discharged.CONCLUSIONS: Endometriosis, although rare, should always be considered in women of reproductive age, presenting with cyclic pain. The diagnosis is, most of the time, difficult and requires a high degree of clinical suspicion. The clinical doctor should be aware that endometriosis can sometimes be multifocal, thus a thorough investigation is required in all cases.