This baby boy was born at 33 2/7 weeks gestational age to a 36-year-old gravida 3, para 3 mother with negative serologies, except for Group B Streptococcus unknown, whose pregnancy was complicated by medication-controlled gestational diabetes and gestational hypertension, and a family history significant for a sibling with LCHADD. The patient was delivered by cesarean section at an outside facility due to concern for serious maternal morbidity associated with preeclampsia, specifically HELLP syndrome. Neonatal resuscitation was unremarkable and the Appearance, Pulse, Grimace, Activity, and Respiration (APGAR)s were 8 and 9 at 1 and 5 min, respectively. The birth weight was 2.46 kg, length 47.5 cm, and head circumference 32.5 cm, which were all appropriate for gestational age. The patient was admitted to the level III NICU at the birth facility. A peripheral intravenous line was placed and 10% dextrose fluids at 150 ml/kg/day were initiated. At six hours of life, based on the significant family history and the maternal HELLP syndrome frequently seen in mothers with gestations complicated by LCHADD, our patient was transported to our level IV NICU for metabolic consultation and further evaluation and management. On admission, serum acylcarnitine profile was consistent with LCHADD given elevation of C16-OH and C18-OH levels with the ultimate diagnosis based on perinatal and family history in conjunction with biochemical and molecular studies (Table 1). Echocardiogram shortly after arrival showed a small patent ductus arteriosus and patent foramen ovale. Enteral nutrition with donor human milk was initiated at 12 h of life. Triheptanoin was initiated at 14 h of life at a dose of 0.5 g/kg/day and advanced 0.5 g/kg every 5-7 days. As feeds were advanced by unit standard of 20 ml/kg/day, he was limited to 23 ml/kg/day of human milk with the remainder of his enteral diet consisting of Enfaport (Chicago, IL, United States), SolCarb (Westbury, NY, United States), and Similac liquid protein fortifier (Abbott Park, IL, United States) formulas (Table 2). His feeds were fortified to 24 kcal/oz on day of life 6. On day 7 of life, the patient started to have new bradycardia and desaturation episodes with two small emeses but a reassuring abdominal examination. Enteral nutrition was continued. On day 9 of life, he had one episode of emesis and then hematochezia. An abdominal x-ray (Figure 1) demonstrated pneumatosis intestinalis and he was subsequently diagnosed with NEC (modified Bell Stage IIa). He was managed conservatively with bowel rest, ampicillin-sulbactam and gentamicin for 7 days, and bowel decompression for 5 days. He was maintained on total parental nutrition (TPN) with 4 g/kg/day TrophAmine, glucose infusion rate ~15 mg/kg/min, and 2 g/kg/day 20% Intralipid. Diet following re-initiation of enteral nutrition consisted of a combination of skimmed and full fat maternal and donor human milk, Enfaport, MCT oil instead of triheptanoin (per parental request), SolCarb, and Similac liquid protein fortifier (Figure 2). He recovered uneventfully and was discharged home on day 33 of life.