MR findings in four patients with MR evidence of congenital cystic dilatation of the ventriculus terminalis were reviewed retrospectively. The ventriculus terminalis is a small cavity of the conus medullaris that forms during embryonic development as result of canalization and retrogressive differentiation. The dilated ventriculus terminalis appears on MR images as a small ovoid cavity with regular margination; intralesional fluid resembles cerebrospinal fluid on all MR sequences. After injection of contrast material, MR imaging shows no enhancement of the cyst or its wall, and thus differentiates congenital dilatation of the ventriculus terminalis from cystic tumors, which occur more frequently in this region.