An unexpectedly high percentage of monozygotic twin pairs is discordant for handedness. Some of these twins show mirror-imaging of several ectodermally derived features. Both features of discordant left-right asymmetry may be caused by relatively late monozygotic twinning, when the original embryo has already lost its bilateral symmetry. Language lateralization is related to handedness and may therefore also be altered during the development of embryological asymmetry in some monozygotic twins. Language lateralization was measured with functional MRI in 12 monozygotic twin pairs who were concordant for handedness and in 13 monozygotic twin pairs discordant for handedness. Lateralization indices were calculated from individual language activation patterns. Correlations were calculated to test intra-pair resemblance for language lateralization. The intra-pair correlation for language lateralization was significant in the handedness-concordant group, but not in the handedness-discordant group. In the handedness-discordant group, five twin pairs were also discordant for cerebral dominance; the other twin pairs of discordant handedness exhibited remarkable similarity in language lateralization. The high intra-pair correlation for language lateralization in the handedness-concordant twins suggests a genetic basis for language lateralization. However, in monozygotic twin pairs of discordant handedness, discordance for language dominance occurs in a significant number of twins. Discordant language dominance may be caused by a relatively late time of splitting of the original embryo, which disrupts the normal development of left-right asymmetry.