Twelve patients underwent appendectomy during pregnancy or in the puerperium. The clinical presentation of acute appendicitis is altered during gestation, and diagnosis becomes increasingly difficult when close to term. Abdominal pain, nausea, and vomiting are important symptoms. Peritoneal signs occur in the right lower quadrant early in pregnancy, but the upper quadrant or entire right side are more common locations, as the appendix is displaced upward by the enlarging uterus. Delay in treatment is common because of uncertainty in making the diagnosis and hesitancy to proceed with surgery. In the group of six patients with perforation, there was one maternal death and a loss of three fetuses. There were no complications in the absence of perforation. Prompt diagnosis is the cornerstone of a good outcome, and early surgical intervention is indicated if acute appendicitis is suspected. Pregnancy is not a reason to delay surgery. We review the literature on this topic and present and analyze principles of management.