Hirschsprung disease associated with enterovirus infection. Enterovirus infection in Hirschsprung disease (HD) has only been observed sporadically, and the pathogenic mechanism remains controversial. We report a child with enterovirus type 71-associated HD. The first manifestation of the patient was a febrile illness followed by hypoventilation associated with constipation at 3.5 months of age. A colon biopsy was performed. A second colon biopsy was performed when the patient was 2.5 years old. Viral antigens were found in the ganglion cells of the specimens of both colon biopsies, and enterovirus-specific polymerase chain reaction products were detected in both specimens. Enterovirus type 71-related HD is an extremely rare disorder, and the present case may provide new insights into the pathogenesis of HD.