Hirsch sprung's disease is a congenital disease of the colon that lacks ganglion cells in the myenteric plexus, responsible for the movement of food in the intestine, which causes chronic constipation or bowel obstruction. HD occurs in approximately 1 in 5,000 live births with a total male: female ratio of 3:1 to 4:1.The diagnosis of Hirsch sprung's disease is not always easy and poses serious medical problem in cases in which part of the diagnostic criteria is missing or unconvincing. The enteric nervous system is a complex of neural network and glia that controls intestinal function. In cases in which this system is absent, the part of the intestine that is affected remains inactive. The patient with a typical history of chronic constipation, physical signs of a bloated abdomen, a large colon with a narrow distal segment in contrast to X-ray examination, with an empty rectal ampoule, does not represent diagnostic problem. Loss of ganglion cells is established after performing a rectal biopsy, offered as an aid in the diagnosis of these cases.