Nocturnal enuresis is a common developmental disorder in children, and primary nocturnal enuresis (PNE) is the dominant subtype. The main purpose of this study was to investigate brain functional abnormalities specifically related to motor response inhibition in children with PNE using fMRI in combination with a Go/NoGo task. Twenty-two children with PNE and 22 healthy children, group-matched for age and sex, took part in this experiment. Although no significant between-group differences in task performance accuracy were observed, PNE patients showed significantly longer response times on average. There were several brain regions with reduced activation during motor response inhibition in children with PNE: the bilateral inferior frontal gyri, right superior and middle frontal gyri, right inferior parietal lobe, bilateral cingulate gyri and insula. Our data indicate that response inhibition in children with PNE is associated with a relative lack of or delay in the maturation of prefrontal cortex circuitry that is known to suppress inappropriate responses. This result might give clues to understanding the pathophysiology of PNE. Hum Brain Mapp, 2012. © 2011 Wiley Periodicals, Inc.