This cross sectional study was conducted in Paediatric Endocrine Outpatient Department of BIRDEM General Hospital, a tertiary care centre in Dhaka, Bangladesh among patients diagnosed with congenital adrenal hyperplasia (CAH) from January 2005 to December 2018. The study was aimed to find out the clinical and laboratory profile of all patients at presentation diagnosed with CAH during the study period. Data were obtained by reviewing the medical records of the patients. Total 102 children with CAH were diagnosed during the study period. Among them 68 were female and 34 were male (female to male ratio of 2:1). Median age was 3.5 month (range 0.2-158 month) and 5.5 month (range 1-108 month) in female and male respectively (p=0.42). Family history was available in 93 patients. Consanguinity was present in 16(17.2%), history of sib death in 12(12.9%), other family members were affected in 8(8.6%). Sixty patient (58.8%) had salt-wasting (SW), 39(38.2%) had simple virilizing (SV) and 3(2.9%) had non- classic form of CAH. Median age of presentation was 2 month (range 0.2-70 month) and 42 month (range 0.8-158 month) in SW and SV group respectively (p=0.001) and 119 month (range 108-152 month) in non- classic group. Common presentations were: genital ambiguity (64.7%), vomiting (46.5%), failure to thrive (41.6%), features of early puberty (precocious pseudopuberty) (24.5%), diarrhea (12.0%). Hyperpigmentation was noted in 49.0% of patients. Among the salt-wasting type in male failure to thrive (FTT) was the most common presentation (83.3%), followed by vomiting (75.0%). In female genital ambiguity was the commonest presentation (97.2%), followed by vomiting (77.1%). Among the simple virilizing type in male early puberty was the commonest presentation (100%) and genital ambiguity was the presenting feature in all the female (100.0%).