Several novel biologics are under development to treat HTG and may provide therapeutic options for FCS in the future.<h4>Objective</h4>We present the challenging care of a 43-year-old man with FCS with apoC-II deficiency and the results of two types of TPE and of investigational TG-lowering biologic therapies.<h4>Results</h4>The patient's lipid profile was consistent with FCS. Here, APOC2 is linked to hyperinsulinemic hypoglycemia, familial, 4.