Twin-twin transfusion syndrome (TTTS) is a serious condition that affects 10% to 15% of twin pregnancies with monochorionic diamniotic placentation. The pathophysiology of TTTS is not completely understood; however, the presence of unbalanced placental vascular communications within a shared circulation has been implicated in its development. The presentation of TTTS is highly variable, and it does not always progress in a predictable manner. Monochorionic twin gestations should, therefore, be monitored for signs of TTTS with serial sonograms starting in the second trimester. Early TTTS can be managed conservatively. However, without intervention, early-onset advanced TTTS is associated with a high perinatal loss rate and risk of severe neurologic impairment among survivors. Limited studies suggest that fetoscopic laser photocoagulation is the best available treatment for advanced TTTS diagnosed in the second trimester. Even with laser therapy, there remains a significant risk of twin demise and neurologic handicap in survivors.