Two children with left open-lip schizencephaly are reported. Cytomegal
ovirus (CMV) infection was demonstrated on the second day of life in P
atient 1; in the other patient CMV infection was observed at the age o
f 4 years. Magnetic resonance imaging revealed polymicrogyria cortex l
ining the clefts. In Patient 2 polymicrogyria was also present in the
corresponding area contralateral to the schizencephaly. Computed tomog
raphy revealed the presence of subependymal periventricular calcificat
ions. Genetic analysis did not demonstrate the presence of EMX2 homeob
ox gene. The possible role of CMV infection in the complex multifactor
ial pathogenesis of schizencephaly is therefore suggested.