To compare the mortality of monochorionic-monoamniotic (MCMA) and monochorionic-biamniotic (MCBA) twin pregnancies, from 14weeks of gestation to 28th day of life, before and after exclusion of major congenital malformations.We conducted a retrospective cohort study in two level 3 maternity units of the Hospices civils de Lyon (France) including all patients with a monochorionic twin pregnancy between January 2013 and December 2020. We excluded TRAP sequences and conjoined twins.A total of 38 MCMA and 658 MCBA pregnancies were included. We showed an increase in overall mortality in the MCMA group compared to the MCBA group (31.6% versus 16.4%, P=0.03) even after exclusion of major congenital malformations (20.3% versus 9.5%, P=0.01). The main cause of mortality found in the MCMA group was the occurrence of unexpected IUGR.MCMA pregnancies have a higher foetal and neonatal mortality rate than MCBA pregnancies even after exclusion of congenital malformations related to the occurrence of unexpected MFIU.