Home parenteral nutrition (HPN) is a life-saving therapy for patients who are not able to use their gastrointestinal tract. There are a number of complications associated with HPN, including metabolic bone disease, intestinal failure–associated liver disease (IFALD), and catheter-related bloodstream infections. We present a case of a 32-year-old HPN patient who initially developed biopsy-proven IFALD (total bilirubin, 2.4 mg/dL) while on long-term HPN. His HPN was initiated due to myopathic intestinal dysmotility and pseudo-obstruction when he was 15 years old. Because of his IFALD, the intravenous (IV) fat emulsion had been reduced and dextrose increased to >500 g/d in the HPN. Although the IFALD improved, he had signs of insulin resistance and struggled with numerous episodes of pancreatitis. His dextrose was decreased and insulin resistance improved, but he began losing weight. As his IV fat emulsion was gradually increased, IFALD worsened and he was switched to mixed-oil (MO) IV fat emulsion (30% soy, 30% medium-chain triglycerides, 25% olive oil, and 15% fish oil). His IFALD improved and total bilirubin normalized (0.4 mg/dL) when switched to the MO lipid. He has been on MO lipid for >8 months (current dose 70 g given 7 days per week) with no more episodes of pancreatitis, normal liver enzymes, and weight gain.