Skip segment Hirschsprung's disease: a rare phenomenon.

Skip segment Hirschsprung's disease (SSHD) involves a skip area of normally ganglionated intestine, surrounded proximally and distally by aganglionosis. We report a case of SSHD in infant with total colonic aganglionosis (TCA). Although SSHD is a rare and controversial condition, it should be considered a definite entity in the evaluation of Hirschsprung's disease.