Tectocerebellar dysraphism is a very rare malformation associated with encephalocele and tectal deformity. This article presents tectocerebellar dysraphism with a solely vermian content of encephalocele and tectal beaking defined by magnetic resonance imaging (MRI) in a 5-month-old girl who was successfully treated surgically and demonstrated excellent prognosis at the 4-year follow-up.
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