BACKGROUND: Heterotopic pregnancy and amniotic embolism are rare conditions that can be challenging to diagnose. To date, there are no cases of heterotopic pregnancy associated with amniotic embolism described in the literature. Therefore, we report the case of a pancreatic heterotopic pregnancy, which led to amniotic embolism and an unfavorable maternal outcome. CASE PRESENTATION: A 20-year-old pregnant woman presented with nausea and abdominal pain refractory to drug treatment. She had an increased lipase of 205 U/L (reference 8 to 78 U/L) and a total abdominal ultrasound with biliary sludge. The pregnant woman had no known risk factors for ectopic and heterotopic pregnancy. The initial diagnostic suspicions were hyperemesis gravidarum or acute pancreatitis. During hospitalization, she fluctuated between periods of clinical improvement and worsening of abdominal pain, but serial laboratory control had a favorable and benign evolution. On the 30th day of hospitalization, the patient developed spontaneous abortion, respiratory failure, and cardiorespiratory arrest. Necropsy showed heterotopic pregnancy in the pancreatic tail, amniotic embolism, and consequent disseminated intravascular coagulation. CONCLUSION: Obstetricians should suspect heterotopic pregnancy in patients with an acute abdomen. The gold standard diagnostic method for this condition is laparoscopy. In the present case, the authors consider that pancreatic pregnancy allowed the introduction of fetal antigens and amniotic fluid into the maternal bloodstream, leading to amniotic embolism and consequent disseminated intravascular coagulation.