Six children with chronic diarrhea and neutropenia, initially referred for evaluation of Shwachman-Diamond syndrome, were found to have no evidence of pancreatic insufficiency. All presented in the spring with a prodromal respiratory illness. Hematologic evaluation was normal except for iron deficiency anemia and neutropenia. Small intestinal biopsies of all children showed inflammation, consistent with chronic enteritis. The children were followed until growth returned to previous percentiles. Diarrhea and neutropenia resolved by 6-month follow-up, and there was no recurrence of the neutropenia at 1 year.