Purpose of Review <p>Monochorionic monoamniotic (MCMA) twin gestation represents the highest-risk form of twin pregnancy and remains a challenging entity in maternal-fetal medicine. Despite substantial advances in prenatal diagnosis, fetal surveillance, and neonatal care, management strategies continue to rely heavily on observational data and expert consensus. This review aims to synthesize contemporary evidence, address misconceptions, and highlight evolving strategies in the management of MCMA twin pregnancies.</p> Recent Findings <p>Improved prenatal detection and standardized delivery planning have significantly reduced perinatal mortality in MCMA gestations over the past few decades. Umbilical cord entanglement is now understood to be nearly universal and poorly predictive of outcome. Contemporary studies suggest that inpatient surveillance may reduce fetal death compared with outpatient management, though definitive evidence is lacking. Planned preterm delivery between 32 and 34 weeks’ gestation has emerged as standard practice, balancing the competing risks of sudden intrauterine demise and neonatal morbidity from prematurity.</p> Summary <p>Optimal management of MCMA twin pregnancy requires early and accurate diagnosis, individualized surveillance strategies, multidisciplinary coordination, and shared decision-making. While consensus supports intensive fetal monitoring and planned preterm cesarean delivery, knowledge gaps remain. Future research should focus on refining risk stratification and identifying which patients derive the greatest benefit from intensive surveillance strategies.</p>