OBJECTIVE To determine the frequency of clinical features of Celiac disease (CD) and Celiac crisis in children. METHODS Case series. METHODS Paediatrics Unit, Sheikh Zayed Medical College and Hospital, Rahim Yar Khan, from September 2009 to September 2010. METHODS Forty children aged between 4 to 13 years of either gender, presenting with complaints of recurrent diarrhea, abdominal distention, severe emaciation and dehydration were included. The information about breast feeding, weaning diets, age of introduction of wheat diets, onset of diarrhea, characteristics and frequency of stools, growth, vaccination status, symptoms in 1st degree relatives, restriction of Gluten diet in the past and anthropometric measures were recorded. Serological tests against anti-Tissue Transglultaminase (anti-tTG) antibodies were obtained in all cases. Upper gastrointestinal endoscopies were performed and multiple biopsies were taken from distal parts of duodenum. RESULTS Among the forty children, twenty four (60%) were females and 16 were males (40%). The mean age was 6.35 ± 2.83 years. Thirty five (87.5%) parents were cousins. Breast feeding was not exclusively given and the Gluten containing weaning diets were given as early as 3.5 months of age. Thirty (75%) children presented with typical sign and symptoms of CD. Celiac crisis presented with profuse diarrhea, severe dehydration; abdominal distention; pedal edema, carpopedal spasm due to tetany; wasted muscles; head drop and inability to stand. The serum TtG antibodies in thirty-eight cases (95%) were above the cut off level of 7 u/ml ranging from 35-99 u/ml. The histopathology of specimens from distal duodenum revealed lesions of M3 type in thirteen (32.5%) and M2 type in eighteen cases (45%). All cases recovered and improved on follow-up after strict adherence to gluten-free diet (GFD). CONCLUSIONS Majority of children with Celiac disease presented with typical symptom, while Celiac crisis was characterized by severe dehydration, weakness and calcium deficiency signs. Most were the product of consanguineous marriages and were given Gluten-containing weaning foods as early as the 4th month of life.