Aim: To investigate the metabolic effects of recombinant human growth hormone (rhGH) in an Alström syndrome patient with growth hormone deficiency. Methods: A 15-year-old Alström syndrome boy with growth hormone deficiency received rhGH therapy for 1 year. Biochemical parameters, including hepatic enzyme levels, lipid profiles, and insulin sensitivity, were measured. Body composition analysis and computed tomography scans of the liver were performed. Results: After 1 year of rhGH treatment, body fat mass, fat infiltration in the liver, and serum lipid profiles had all decreased. Insulin sensitivity and acanthosis nigricans improved. Conclusion: rhGH therapy might have beneficial effects on body composition, liver fat content, lipid profiles, and insulin resistance in Alström syndrome patients, with improvement of the glucose homeostasis.