Amniotic fluid embolism (AFE) is a rare obstetric emergency with high maternal morbidity and mortality. Despite ongoing research, the pathogenesis of AFE remains unresolved, and AFE is a diagnostic and therapeutic challenge. We present a case report of suspected AFE and disseminated intravascular coagulation occurring during caesarean delivery in a pregnancy with oocyte donation and intracytoplasmic sperm injection. Awareness of the diagnosis, rapid initiation of treatment and the use of rapid tests of haemostasis in targeted coagulopathy treatment may improve clinical outcome.