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Encephalocele means if meninges and brain tissue protrude out of the cranium. There are different types of encephalocele. The occipital encephaloceles are the most common type. Craniocervical junction and upper cervical spine abnormalities can rarely be associated with occipital encephalocele. We discuss this case because there is rare association between torcular encephalocele and proatlas anomalies.