This study identified a de novo heterozygous frameshift variant in HNRNPK (c.504_507del; p.Lys168AsnfsTer35) in an 18 + 1 week fetus with increased NT (3.4 mm) and NF (9 mm), contributing to the prenatal phenotypic spectrum of AUKS. The gene discussed is HNRNPK; the disease is Au-Kline syndrome.