Endodermal cysts are rare congenital lesions of the central nervous system (CNS), but well known, located mainly in the spinal subdural space, its intracranial location in the cerebral parenchyma is so very rare. The best treatment is complete removal without leaving any residue. We report the case of a right parietal intraparenchymal endodermal cyst and recall this rare lesion.

A 3-year-old child with no specific pathological history admitted for generalized tonic-clonic epileptic seizures having evolving for 3 months before admission, aggravated by a 3/5 left hemiparesis. A cerebral MRI showed a voluminous right temporo-parietal cystic process. The cyst was completely removed with excision as complete as possible of the cystic wall. The histological examination showed an endodermal cyst.

Epileptic seizures have disappeared aided by antiepileptic treatment, the neurological deficit has regressed completely.

The endodermal cyst, despite its rarity, must be evoked facing any intraparenchymal cystic lesion, and complete surgical excision is the treatment of choice.