This study addressed the clinical and construct validity of the Behavior Rating Inventory of Executive Function. (BRIEF: Gioia, Isquith, Guy, & Kenworthy, 2000), a questionnaire designed to tap behavioral aspects of executive functions in children. BRIEF profiles in early treated phenylketonuria (PKU; n = 44), early treated hydrocephalus (n = 45), frontal focal lesions (n = 20) and controls (n = 80) were examined. Clinical validity was supported through significant between-group comparisons, especially between the frontal focal lesion group and other groups. To examine construct validity, raw scores on cognitive executive function measures including the Contingency Naming Test (CNT), Rey Complex Figure (RCF), Tower of London (TOL), and Controlled Oral Word Association Test (COWAT), were correlated with BRIEF scale scores. Few significant correlations were found, indicating cognitive and behavioral measures appear to tap different constructs within the executive function domain. A dissociation was found between behavioral and cognitive impairments in the frontal as opposed to PKU and hydrocephalus groups. This is discussed in relation to underlying pathology, the cognitive measures used, and possible limitations in the BRIEF's usefulness for measuring behavioral executive dysfunction in groups only mildly affected by neurological compromise.