A case report of a 23-week premature infant with short bowel syndrome who developed severe essential fatty acid deficiency after intravenous lipid emulsions were withheld and then suboptimally dosed. Treatment with 100% fish oil intravenous lipid emulsion at 1.5 g/kg/d over 16 weeks normalized the triene:tetraene ratio, transaminases, and conjugated bilirubin.