An 11 months 22 days old girl presented with a history of watery diarrhoea since birth, failure to thrive, and developmental delay. Her diagnosis was congenital chloride diarrhoea (CCD) with raised level of chloride (>90 mmol/L) in stool in the absence of cystic fibrosis. Management of CCD included replacement of NaCl, KCl, and correction of dehydration. Diarrhoea of the patient was resolved with Captopril, which was initially provided to the patient for managing heart failure. To our knowledge, this is the first reported case of CCD that shows the beneficial effect of Captopril. Therefore, we suggest that further study is warranted as to the potential for Captopril as additional option in the treatment for CCD. We present this case report with the informed consent of the patient's guardian.