Cytomegalovirus(CMV) is the most common cause of congenital viral infections. CMV infection occurs in 0.4% to 2.4% of all live births. CMV causes thin cerebral cortices, diminished volume of white matter, and delayed myelination, bringing on encephalopathy, which may be manifested as seizures in some cases. CT findings in CMV encephalopathy present as irregular intracranial calcifications of the periventricular area. Recently, there are increasingly more reports about MRI findings in CMV encephalopathy and common findings of the encephalopathy are periventricular cysts and dilated lateral ventricles. We experienced a case of congenital CMV encephalopathy with patchy, nodular lesions of the periventricular area on magnetic resonance imaging(MRI). We report this case with a review of associated literature.