In vivo, ex vivo and modelling studies suggest that arterio-arterial anastomoses (AAAs) protect against haemodynamic imbalance in monochorionic twins and thus the development of TTTS. We report the acute onset of severe TTTS at 34 weeks' gestation in a patient with an antenatally visualized AAA which was shown at injection studies to have been obliterated, presumably by thrombosis. Computer modelling with the relevant clinical data confirmed that occlusion of the AAA alone was sufficient to reproduce the clinical manifestations. A study of the vascular configuration of AAA in the fixed placenta suggested that its small diameter and turbulent flow may have contributed to its occlusion. This case report shows that the unmasking of unbalanced AVA configurations by occlusion of a protective AAA can manifest as TTTS.