Acute appendicitis during pregnancy is one of the most common non-obstetric acute abdominal conditions, and it can occur at all stages of pregnancy, most frequently within the first 6 months of pregnancy. Due to the special physiological situation of pregnancy and the changes in the position of the appendix, the clinical manifestations of acute appendicitis during pregnancy are atypical, and highly variable at different stages of pregnancy. In the early pregnancy, the clinical manifestations are similar to those in the non-pregnancy period, with the typical sign of the localized tenderness over McBurney's point; in the middle and late pregnancy, the appendix becomes displaced upwards laterally and backwards, and the symptoms and signs are nonspecific, which likely leads to misdiagnosis and missed diagnosis, and thereby cause appendiceal perforation or gangrene, fetal abortion, premature delivery, or even endanger the life of mother and baby due to delayed treatment. In addition, laboratory findings are nonspecific, and auxiliary examinations such as imaging are required for diagnosis. Both ultrasound and MRI are safe and effective imaging diagnostic modalities, in which, ultrasound has the characteristics of convenience, rapidness and safety, and is the first choice of imaging examination at the initial diagnosis. Surgery is the primary treatment method, and should be ideally performed within 24 h of the onset of symptoms, so as to reduce the maternal and infant mortality and incidence of the associated complications. According to the surgical approaches, the surgical procedures include traditional open surgery and laparoscopic surgery. However, the selection of surgical procedures at present should be based on the general factors that include the existing expertise, surgeon’s experience, medical facilities, disease status, and patients’ intention. Laparoscopic surgery has been proven to be safe and effective, and open surgery is still the dominant choice.