Here, we describe a de novo missense mutation (c.560A>G) in the human Fzr1 gene that predicts an aspartate‐to‐glycine substitution (p.Asp187Gly) in the Cdh1 protein, which results in prenatal microcephaly, psychomotor retardation, and severe epilepsy. This evidence concerns the gene FZR1 and epilepsy.