We report on the treatment failure of low molecular weight heparin (LMWH) for anticoagulation in a pregnant woman that underwent artificial mitral valve replacement 10 years prior to her pregnancy. Until she became pregnant warfarin was administered for anticoagulation, but due to the often mentioned increased risk for warfarin-induced maternal and fetal complications, at gestational week 5 the anticoagulation regimen was switched to subcutaneous application of low molecular weight heparin. At gestational week 24 our patient developed acute life-threatening pulmonary edema and hemodynamic instability due to acute mitral valve thrombosis and underwent emergency valve re-replacement with a biological porcine valve. She recovered uneventfully and gave birth to a healthy child at gestational week 35. In addition to our case presentation we review the sparse evidence in the literature regarding anticoagulation in pregnant women with mechanical heart valves and discuss the rational of different anticoagulation regimens with regards to maternal and fetal outcome. Special consideration is directed towards LMWH administration as an alternative to oral anticoagulation during pregnancy in women with mechanical heart valves.