A literature review revealed 13 previously reported pediatric cases, most of which were diagnosed after the age of 11 years and were frequently associated with systemic lupus erythematosus or other autoimmune disorders.<h4>Conclusion</h4>GPIHBP1 autoantibody-related HTG is important and potentially treatable severe pediatric HTG, representing an early manifestation of autoimmune dysregulation that requires an accurate diagnosis and longitudinal surveillance. Here, GPIHBP1 is linked to systemic lupus erythematosus.