A moderately retarded Japanese boy, with a normal male karyotype (46,XY), was diagnosed to have a subcortical band heterotopia or double cortex syndrome. The band heterotopia was relatively thick compared with that of other patients reported. On T2-weighted coronal MR sections, there were numerous radial linear structures between the cortex and the band, probably representing the trace of radial fibers. He had no family members with seizures or mental retardation. Over 50 described patients with this malformation have been female except two patients briefly mentioned by several investigators. Band heterotopia or the double cortex syndrome is inherited as a sex-linked dominant condition. Affected mothers may have affected daughters or sons with lissencephaly, suggesting a link between these disorders. This is the first detailed description of a male with band heterotopia.