Familial LPL deficiency is a rare autosomal recessive disorder which is characterized by primary hyperchylomicronemia due to homozygous or compound heterozygous mutations of LPL gene [5] as well as to homozygous mutations of APOC2,GPIHBP1, APOA5 or LMF1 genes [6]–[8]. The gene discussed is GPIHBP1; the disease is familial lipoprotein lipase deficiency.