Follow-up data showed that HTG normalized in 28% of patients (11 out of 39) but persisted mildly in the remaining 72% (28 out of 39).<h4>Conclusion</h4>Mutations in the <i>GPD1</i> gene should be considered in children with hepatomegaly, hepatic steatosis, and HTG. The gene discussed is GPD1; the disease is fatty liver disease.