Since boys are more commonly diagnosed with Attention Deficit Hyperactivity Disorder (ADHD) than girls, the majority of theories and published research studies of ADHD have been based on samples comprised primarily (or exclusively) of boys. While psychosocial impairment in girls with ADHD is well established, the neuropsychological and neurobiological basis of these deficits is less consistently observed. There is growing evidence that boys' and girls' brains develop and mature at different rates, suggesting that the trajectory of early anomalous brain development in ADHD may also be sex-specific. It remains unclear, however, whether earlier brain maturation observed in girls with ADHD is protective. In this review, we outline the current theory and research findings that seek to establish a unique neurobiological profile of girls with ADHD, highlighting sex differences in typical brain development and among children with ADHD. The review highlights findings from neurological, neurocognitive, and behavioral studies. Future research directions are suggested, including the need for longitudinal neuroimaging and neurobehavioral investigation beginning as early as the preschool years, and continuing through adolescence and adulthood, with consideration of identified sex differences in the development of ADHD. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:276–284.