We report on five patients who presented with intractable diarrhea sta
rting during the first days of life. The patients belonged to four fam
ilies of Iraqi Jewish origin. Autosomal recessive inheritance is sugge
sted by parental consanguinity in three families and recurrence in ano
ther sib in one family. The patients were all born after uneventful pr
egnancy and labor, with birth weight in the normal range. There were n
o dysmorphic features. Three patients were breast fed. Diarrhea starte
d between the first and eighth day of life. Diarrhea was of the secret
ory type. No pathogen was cultured from the stool. Jejunal biopsies pe
rformed on all patients ranged from normal to severe partial villous a
trophy. The patients received different drug regimens with no benefici
al effect and all are dependent on TPN. These findings and the common
ethnic origin of the patients suggest that these patients have the sam
e syndrome of congenital intractable diarrhea. No similar cases are kn
own in other ethnic groups in Israel, suggesting a possibility of high
gene frequency among the Jews of Iraqi origin.