In a fetus diagnosed with fetal hydrops, multicystic dysplastic kidneys, cardiomyopathy, lung hypoplasia, retrognathia, and hydrocephalus in the 18th week of pregnancy, we performed an sES that showed a known homozygous disease-causing variant of the canonical donor splice site (c.608 + 1 G > A) in the CDK10 gene. The gene discussed is CDK10; the disease is Hydrocephalus.