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Spontaneous heterotopic pregnancy (HP) is a very rare and fatal condition that could result in significant maternal morbidity and mortality if prompt diagnosis and appropriate interventions are not instituted at the right time. Unfortunately, this life-threatening condition may be easily misdiagnosed in early pregnancy due to its rarity, vague clinical presentation and the presence of an intrauterine pregnancy which may confuse an inexperienced clinician. The aim of management is to excise the ectopic gestation while preserving the intrauterine pregnancy, if alive. We present a case of spontaneous HP that had complete excision of the ectopic gestation and a live delivery of the intrauterine pregnancy at term.