Introduction: Monochorionic monoamniotic (MCMA) twins are rare, and information is lacking on pregnancy outcomes from the first trimester onward. This study compares the pregnancy and neonatal outcomes between MCMA and monochorionic diamniotic (MCDA) twin pregnancies from the first trimester onward. We also report on the outcomes of MCMA twin pregnancies continuing after 28 weeks and on the neurodevelopmental outcomes of MCMA twins. Methods: A retrospective single-center cohort study of MCMA and MCDA twin pregnancies followed from the first trimester with a fortnightly ultrasound scan. We excluded pregnancies with a major anomaly diagnosed on the first-trimester ultrasound scan. MCMA twin pregnancies were offered inpatient monitoring from 28 weeks onward, and an elective cesarean section was advised between 32 and 33 weeks. MCDA pregnancies were managed as inpatients only if medically indicated, and an elective birth between 36 and 37 weeks was recommended. Results: We analyzed the outcomes of 52 MCMA and 671 MCDA twin pregnancies. In MCMA twins, the fetal and neonatal survival rate was 81/104 (78%) versus 1,192/1,342 (89%) in MCDA twins (p = 0.016). Double intrauterine demise (IUD) was more common in MA than in DA pairs (13% vs. 3%) (p = 0.002). No IUD occurred in the 40 MCMA pregnancies that continued after 28 weeks, but five women (13%) required an urgent cesarean section for fetal distress. Thirty-eight of 52 eligible infants (73%) underwent a neurodevelopmental assessment. Thirty-three (87%) had cognitive and motor development scores within normal limits. Four infants had mild cognitive or motor impairment, and one infant was diagnosed with spastic diplegia. Conclusion: MCMA twins are associated with decreased survival rates compared to MCDA due to increased rates of double IUD. After 28 weeks, about one in eight women required an urgent delivery for fetal distress. Most infants had normal neurodevelopmental outcomes.