BACKGROUND AND OBJECTIVE: Hirschspnmg's disease (HD) is a congenital defect in pediatric patients, which is manifested by bowel obstruction in neonates or constipation in children. The gold standard of diagnosis is absence of ganglionic cells in myenteric neural plexus and submucosa of bowel. With regard to barium enema is a simple, less invasive and more available method than rectal biopsy, diagnostic evaluation of barium enema compared with rectal biopsy (as a diagnostic method) in HD is important.METHODS: In a cross-sectional study, 84 children with clinical manifestations of HD that referred to Boo-Ali Sina hospital of Sari, Iran were assessed from 2004 through 2007. Barium enema and rectal biopsy were carried out for all cases. Patients divided into three age groups as follows: under 1 month. 1- 12 months, more than 12 months Patient with previous history of anorectal surgery and negative pathology of HD excluded from the study. Sensitivity, specificity, positive predictive value and negative predictive value, of barium enema were calculated for identifying of the HD.FINDINGS: From 84 studied children, 28(33.3%) were girl. The mean age of the children was 26.1 months. The sensitivity, specificity, positive predictive value and negative predictive value of barium enema for diagnosis of the HD were 63.5%, 78.1%, 82.5%, and 56.8%, respectively. Diagnostic value of barium enema was calculated according to age groups mentioned above, that; there were significant differences among them (p<0.05).CONCLUSION: The results of the study showed that the validity of barium enema for identifying of the HD is completely age related. This validity in specificity and positive predictive value are more significant than in sensitivity and negative predictive value. Therefore, it is recommended considering all three aspects of clinical manifestations, barium enema and rectal biopsy in diagnosis of HD. of course, the diagnostic value is related to rectal biopsy.