Heterotopic pregnancy (HP) refers to a pathological condition where intrauterine pregnancy coexists with an ectopic pregnancy. The natural occurrence rate of HP is relatively low, but its incidence has notably increased with the widespread use of assisted reproductive technologies. Diagnosis requires multidisciplinary collaboration involving gynecology, reproductive medicine, ultrasonography, and obstetrics. Comprehensive evaluation based on medical history, symptoms, clinical signs, and diagnostic investigations is essential. Once HP is diagnosed, management primarily focuses on ensuring the patient’s safety, addressing the ectopic pregnancy at different locations in an individualized manner, and optimizing the prognosis of the intrauterine pregnancy. Postoperative perioperative care and pregnancy management are crucial following surgery for HP.