Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an enzymatic defect of the fatty acid (FA) beta oxidation pathway. In catabolic states, such as labor and early postpartum period, patients are potentially prone to metabolic decompensation and subsequent rhabdomyolysis with increased risk for myoglobinuria and renal insufficiency. We report a 21-year-old primigravida with a previously characterized VLCAD deficiency, who experienced frequent and unprovoked episodes of rhabdomyolysis before pregnancy. As there was no published experience to guide her management, a detailed multidisciplinary care plan was established to minimize the potential morbidity. Although there is little known about the antenatal course of gravidae affected by VLCAD, we predicted that placental and fetal beta-oxidation in an unaffected pregnancy may temporize or even improve maternal FA beta-oxidation. Consistent with our prediction, we observed a significant clinical and biochemical improvement throughout her pregnancy, and she delivered vaginally with an uncomplicated postpartum course. We conclude that although VLCAD deficiency can present a therapeutic challenge during pregnancy, the beneficial placento-maternal metabolic interactions and the implementation of a proper peripartum management reassure a successful antenatal and perinatal outcome.