This review covers the clinical presentations, treatments, and outcomes of cornual heterotopic pregnancies reported in the literature. Infertile women with a history of ectopic pregnancy, tubal surgery, or disease are at increased risk for cornual heterotopic pregnancy when they undergo in vitro fertilization. Women who have undergone bilateral salpingectomy also seem to be predisposed to this condition when they undergo in vitro fertilization. We recommend that these patients be followed up closely after a successful in vitro fertilization cycle with monitoring of serum beta-human chorionic gonadotropin levels and serial transvaginal ultrasonography because of the high associated morbidity. Laparotomy remains the treatment of choice for rupture of a cornual heterotopic pregnancy. In the absence of cornual rupture, however, medical management is an option that eliminates the risk of surgery and anesthesia and results in outcomes similar to those associated with surgical treatment. Currently there is insufficient evidence to recommend any single treatment modality, and the decision should be based on such factors as clinical presentation, surgeon's expertise, side effects, overall cost, and the patient's preference.