To date the APOC3 variant c.11G > A–p.(Arg4Gln) found in our patient and classified as of uncertain clinical significance according to ACMG guidelines, has never been described as associated to severe hypertriglyceridemia and if its pathogenicity resulted as GOF, the patient would be considered as carrier of a rare pathogenic variant. Here, APOC3 is linked to hypertriglyceridemia.