Introduction: Glutaric acidemia type 2 is a rare inherited disorder with deficiencies in electrontransfer flavoprotein (ETF) and ETF-dehydrogenase resulting in inability to metabolize certain fats and proteins. Case presentation: Baby girl, born at term, with a birth weight of 2.56kg, to parents with thirddegree consanguinity who experienced two miscarriages and two neonatal deaths on day 4 of life following hypoglycemia, was evaluated for possible metabolic disorder. Baby was noted to have pale skin, hypotonia and reduced activity since birth. USS abdomen was normal on day 4. On day 6, She developed metabolic crisis with hypoketotic hypoglycemia (blood sugar=11mg/dL), hyperammonemia (412micromol/L- could be partly due to pre-analytical issues), elevated lactate(8.2mmol/l) and seizures following glucose correction. 2D echocardiography on day 8 excluded cardiomyopathy . On day 12 EEG demonstrated multifocalepileptiform discharges with abnormal background activity . Neuroimaging revealed multifocal ischemic changes. She required noninvasive ventilation from day 13-20 for respiratory distress. Baby is now 21 days old and is stable on nasal prong oxygen. Baby had normal/alkalotic pH with decreasing lactate levels (11mmol/l to 0.6mmol/l). Her aminoacid profile, galactose and biotinidase levels were normal. Mild elevation of acylcarnitine C4, C5, C6, C7 , C8, C10, C5DC, C12, C14:1, C14 was suggestive of glutaric acidemia type 2. We are awaiting urine organic acid analysis and genetic diagnosis. Baby is treated with multivitamin containing riboflavin, carnitine and ammonia scavengers. Conclusion: Metabolic disease should be suspected in neonates with family history of consanguinity , recurrent miscarriages and early neonatal deaths due to hypoglycemia.