Large midline persistent parietal foramina with occipital encephalocele and abnormal venous drainage

Enlarged persistent parietal foramen is rare congenital skull defect and associated anomalies like underlying encephalomalacia, and venous malformations are known. We here report a very rare association with persistent occipital foramina and occipital encephalocele. This patient presented later in life with complaints of headache and seizure. Basic clinical examination like palpation of scalp was helpful in diagnosing this rare condition. Radiological investigations later revealed an array of associated congenital abnormalities like hypoplastic inferior sagittal sinus, which is even rare finding in a single case.