BACKGROUND: The aim of this study was to describe clinical features of children and adolescents with celiac disease (CD) and to assess if the presenting symptoms vary with age and sex. METHODS: In this cross-sectional study, a total of 65 known cases of patients with CD, both males and females whom diagnosed in Namazi Hospital, a large referral center in south of Iran, by census were studied in 2014. A detailed data about clinical features and associated disorders, for all patients were assessed based on their medical records and also clinical examination. Two groups were made as per the age of the patients at the time of diagnosis, groups I (less than or equal to 10 years old) and II (11-18 years old). RESULTLTS: The mean age at diagnosis was 8.11 years. Growth failure and abdominal pain were the most frequent presenting features. Diarrhea was more common in females (36.8% vs. 18.5%, P=0.1). The most common presenting features in group I were growth failure, abdominal pain and anorexia and in group II were growth failure, short stature and abdominal pain. All cases of convulsion were male (P=0.03). Both abdominal distention (46% vs. 18.5%, P=0.02) and anxiety (34.2% vs. 11.1%, P=0.03) were more common among female participants. The most frequently associated disorder was diabetes mellitus type 1 (5 patients). CONCLUSIONS: CD should be considered in children and adolescents with growth failure or short stature, even in the absence of gastrointestinal symptoms, especially diarrhea.