While the prenatal phenotype of fructose‐1,6‐bisphosphatase deficiency is not well characterized, we hypothesize that the FBP1 gene variant detected in Case 82 could explain the increased nuchal translucency, as inborn errors of metabolism are known to cause nonimmune hydrops fetalis (Sparks et al. 2020). Here, FBP1 is linked to hyperinsulinemic hypoglycemia, familial, 4.