Background: Celiac disease (CD) is a chronic autoimmune enteropathy. It results from genetic predisposition and exposure to gluten containing food. The prevalence and presentation of CD varies among populations. Purpose: This study aimed to describe the prevalence and clinical characteristics of CD in children in Bahrain. Methods: We retrospectively reviewed the medical records of children diagnosed with CD in the pediatric department, Salmaniya Medical Complex, Bahrain, in 1988-2018. Their clinical, biochemical, serological, and histopathological findings were documented. Adherence to the recommended gluten-free diet (GFD) was assessed. Results: Of 86 patients with CD, 67 were included. The CD prevalence was 0.02%. A significant increase in prevalence in the last decade was observed (P < 0.0001). Thirty-eight patients (56.7%) were males. The median (interquartile range) age at presentation was 4.45 (5.8) years. A family history of CD was positive in 13 (30.2%) out of 43 patients. Pallor and failure to thrive were the most common presentations. The most frequent associated disease was irondeficiency anemia in 23 (69.7%) patients. Positive serology was found in 32 (71.1%) of 45 patients. Marsh-Oberhuber type III was found in 16 (45.7%) of 35 patients. Seropositive patients were significantly older (P = 0.025) and had more severe duodenal histology (P = 0.002). Adherence to a GFD was poor in 27 (64.3%) patients. Conclusion: This study revealed a significant increase in CD prevalence over the last decade. Atypical presentations were frequent. Most patients had poor adherence to a GFD.