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Spontaneous heterotopic pregnancy causing tubal rupture in a patient with intrauterine device in-situ spontaneous heterotopic pregnancy (HP) is a rare but life threatening condition. A case of 27 years old patient who admitted to our emergency gynecology clinic for acute abdominal pain is presented. The transvaginal ultrasound revealed two embryos with cardiac activity; one intrauterine (8 week and 3 day) , and the other one in the left adnexa (7 week and 3 day) and an intrauterine device (IUD) in situ. The patient had left salpingectomy with laparotomy and after the fifth hour of operation she had spontaneous abortion of intrauterine pregnancy. We reported a case of heterotopic pregnancy and relation with IUD and a brief review of the literature.