Pregnancy complicated with acute appendicitis (AA) and appendiceal endometriosis (AE) is a rare condition whose frequency ranges between 3 and 8 deliveries per 10,000 [1]. The case of a pregnant woman with both AE and immunohistochemistry-confirmed AA is described. A series of cases of AE in pregnancy collected from Ovid, Medline, and PubMed Plus electronic databases, as well as University of Pennsylvania Biomedical library archives, was then compared with pregnancies complicated with AA in general.The patient presented to the emergency room complaining of worsening right lower quadrant pain associated with anorexia, nausea, and vomiting over the last 24 h. She reported no fever, urinary symptoms, or vaginal bleeding. She was a 21-year-old African American woman, gravida 3, para 1, who had 1 abortion and was in the 12th week of a pregnancy. Her vital signs on admission were the following: pulse rate, 98 beats per minute; breathing rate, 18 breaths per minute; temperature, 37.2°C; systolic blood pressure, 105 mm Hg; and diastolic blood pressure, 60 mm Hg. She manifested right lower quadrant tenderness with positive rebound and voluntary guarding. There was no rigidity and no tenderness in the costovertebral angle. A gynecologic examination revealed an enlarged uterus, a closed cervix with no bleeding, and tenderness on the right side with no evident masses.Her white blood cell count was 14,700/mm 3 and the results of the comprehensive metabolic panel and urine analysis were normal. An abdominal ultrasonographic evaluation confirmed a viable 12-week intrauterine pregnancy but the appendix was not visualized.The patient was taken to the operating room with the diagnosis of a 12-week intrauterine pregnancy and presumed AA. A 4-cm skin incision was made in the right lower quadrant just above the McBurney point, revealing an edematous appendix with no perforation. Appendectomy was performed without complications and the patient was discharged home 2 days later.Hematoxylin-eosin-stained slides showed acute transmural appendicitis with peritonitis, and the appendiceal wall had multiple foci of endometrial implants with acute inflammation. A panel of immunohistochemical stains, including cytokeratin (CK)-7, CK20, estrogen receptor, and CD10, confirmed that the intramural glands and the appendiceal mucosa were of different nature, as the former reacted as endometrial mucosa whereas the latter reacted as a colonic-type mucosa (Fig. 1). Table 1 shows that 27% of these AE cases were complicated by perforation at the time of surgery; that there was no