Introduction:Celiac disease typically presents with symptoms of malabsorption,but extraintestinal manifestations are increasingly reported.Aplastic anemia as the mode of celiac disease presentation is extremely rare in children.Case presentation:We report a 2-year-old boy who presented with loose stools,loss of appetite,and bicytopenia with severe aregenerative normocytic anemia.Investigations,including bone marrow aspirate and biopsy,revealed aplastic anemia.Screening for malabsorption showed increased plasma concentrations of anti-transglutaminase and anti-gliadin antibodies.A duodenal biopsy confirmed the histologic features of celiac disease.The child received a packed red cell transfusion and was started on a gluten-free diet,with a very good prognosis and normalization of both his blood and histological parameters.To the best of our knowledge,our report is the sixth pediatric case in the literature.Conclusion:Screening for celiac disease should be performed in children with unexplained hematological abnormalities such as aplastic anemia with or without gastrointestinal symptoms.