A 65-year-old man with progressive muscle weakness and liver dysfunction following massive small bowel resection showed myriad lipid-filled vacuoles in type I muscle fibers. Carnitine was significantly decreased in muscle, serum and urine. Carnitine supplementation was followed by clinical improvement and decreased lipid droplets in biopsied muscle. He had been receiving carnitine-deficient total parenteral nutrition. This form of carnitine deficiency may be due to a defect in carnitine biosynthesis, as well as dietary carnitine deficiency.