Background: Euglycaemic ketoacidosis is a rare type of metabolic acidosis, with increased production of ketonic bodies and normal serum glucose. When it occurs during pregnancy, it might lead to adverse maternal and fetal outcomes. Pregnancy itself is a diabetogenic state that might predispose the patient to develop ketoacidosis following even relatively short periods of starvation and as such, a high index of suspicion is required for the diagnosis to be made and for the management plan to be established. Case presentation: We present a case of euglycaemic ketoacidosis in a twin pregnancy, which occurred in the second trimester, necessitating prompt and aggressive management, that eventually required early delivery due to the development of preeclampsia. Discussion/Conclusion: The physiology and pathology of euglycaemic ketoacidosis during pregnancy and the management required to avoid the potential maternal and fetal risks that such a condition might be complicated with.