A systematic study of celiac disease in a defined population of Arab children has not been previously reported. We therefore performed a prospective study to determine the incidence and clinical presentation of celiac disease in Jordanian children. A total of 34 (12 boys and 22 girls) cases were diagnosed over a period of 36 months. Nine cases were clustered in three families. The incidence was calculated to be 1:2,800 live births. The mean age at presentation was 4.6 years (range 0.3-13), but it was 8.4 years at the time of diagnosis. Diarrhea was the main initial symptom in 15 (44%) patient. Other signs and symptoms included recurrent abdominal pain in five patients (14%), short stature in four (12%), abdominal distension in three (9%), constipation in three (9%), pallor and recurrent mouth ulcers in two patients each. At the time of diagnosis, 24 (71%) had hypochromic microcytic anemia, and nine (26%) had rickets. The relatively high incidence of celiac disease may be related to the large wheat consumption in this population (135 kg/head/year), and the late onset could be related to the widespread prevalence of prolonged breast-feeding in Jordan (12 months) and late introduction of cereals (6.2 months) in children with late presentation. The delay in diagnosis might be due to the presence of other diseases clinically resembling celiac disease in our community.