A 33 year old woman had an uneventful pregnancy and delivery. Newborn screening in her daughter was positive for Carnitine Uptake Defect (CUD). Investigations in mother revealed low total blood carnitine of 2.5 μM and a free carnitine of <1 μM. Medical history and physical examination were unremarkable. Investigations for secondary causes of hypocarnitinemia included an acylcarnitine profile noting an elevation in Glutaryl-carnitine at 1.06 μM (<0.06) and urine organic acids revealing elevations in glutaric acid and 3-hydroxyglutaric acids, thereby securing a diagnosis of GA1. Carnitine supplementation was initiated at 70 mg/kg/day. Molecular analysis of the GCDH gene is underway with results pending. This is the first reported case of an asymptomatic mother with GA1 ascertained by newborn screening of CUD in her unaffected child. Newborn screening has allowed us to detect asymptomatic mothers affected by inborn errors of metabolism, thereby forcing us to shift our paradigms with regards to the pathophysiology and management of these conditions.