Introduction: We report an isolated pedigree in which a consanguineous couple had twin sons with Dandy-Walker malformation (DWM). The mother is similarly affected with the disorder.
Discussion: DWM is an abnormality of the central nervous system, which leads to hydrocephalus and is associated with other abnormalities.
Conclusion: Inheritance of the disorder remains controversial, with the majority perceived to be sporadic cases. This report suggests an autosomal inheritance.
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