Résumé : BACKGROUND: Monoamniotic twinning is a rare event with a high perinatal mortality rate from cord accidents. To date, only a few cases have been reported in which cord entanglement was diagnosed with Doppler ultrasonography. CASE: In a monoamniotic twin pregnancy, umbilical cord entanglement was diag- nosed at 20 weeks' gestation by color flow Doppler ultrasonography. The entanglement was associated with growth discordance between the twins and subsequent death of the smaller fetus. CONCLUSION: The rarity of this condition hinders our understanding of the pathophysiology and development of management protocols.