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Background: CAH Congenital adrenal hyperplasia (CAH) is a genetic disorder, when confronted with the unexpected diagnosis of CAH, the parents experience severe stress. This study assess the stress among parents of children having CAH 21 hydroxylase deficiency and find the association between stress and selected baseline variables. Method: The research design selected for this study was descriptive design. The setting selected was the OPD at Indira Gandhi Institute of Child Health Hospital, Bangalore. Parents of children affected with CAH 21 hydroxylase deficiency were selected for interview schedule to assess the level of stress by them. The collected data were analysed using descriptive and inferential statistics. Results: The findings of the study showed that the parents had severe financial stress. The parents having low income, low educational level had significantly high stress (P<0.001). Parents having more than one child affected and birth order of child = 2 showed more stress and significant at p<0.01 level of significance. The Parents who had not received counselling also elicited more stress at p<0.01 level of significance. Conclusion: These findings of the study clearly point out that CAH 21 hydroxylase deficiency is a stressful event faced by the parents. Positive attitude towards stress is present but needs to be better handled. [ABSTRACT FROM AUTHOR]