Introduction: Congenital chloride diarrhoea (CCD) is a rare autosomal recessive condition. The primary defect is a mutation at the solute carrier family 26-member 3 (SLC26A3) gene. The most common mutation in the Arabian Peninsula is GLY-187-Stop. The pathological mutation results in a non-functioning Cl-/HCO3 exchanger in the ileum and colon. Renal manifestations are common in CCD cases. The incidence of CKD was 28% in the Finnish series and 24% in the Saudi cohort. Few reports have described the outcomes of renal transplantation in CLD patients. Limited information about the outcomes of renal transplants in CCD is available in the literature. 
Method: We report 7 consecutive CCD cases with end-stage renal disease (ESRD) who were referred to a tertiary renal transplant unit between 2009 and 2021. Data was collected retrospectively from medical records. We collected baseline demographic data for each patient, transplant characteristics, immunosuppression, biopsy findings, hospital admissions, kidney function, and electrolytes. 
Results: All patients were diagnosed clinically with CCD during their 1st year of life and developed ESRD due to recurrent dehydration episodes secondary to CCD. The median age for reaching ESRD was 12 years (range 7-21). Fifty-seven per cent of the participants were male. Three patients were on hemodialysis, two were using peritoneal dialysis before renal transplantation, and two were transplanted preemptively. Two patients received renal grafts from deceased donors, while the remaining had living donor grafts. Median post-renal transplantation follow-up was 4.7 yr (1.25 to 13.5). Two patients (28%) lost grafts, one due to recurrent hypovolemic pre-renal acute kidney injury (AKI) + chronic T cell-mediated rejection (TCMR) and the other due to severe interstitial fibrosis and tubular atrophy (IFTA). Five patients had functioning grafts till the follow-up period. Three patients had an intolerance to MMF due to worsening diarrhoea. Two patients were converted to Azathioprine, and the MMF dose was reduced in one case. All patients, apart from one, had nephrocalcinosis on biopsy. Most patients had graft rejections. No study has linked immunological damage to CCD; however, these patients have poor compliance with chronic fluid and electrolyte replacement for CCD, which could extend to the immunosuppressive medications. Interstitial fibrosis and tubular atrophy (IFTA) was reported in earlier biopsies in most cases. Arterial hyalinosis was also reported in some cases.  
Conclusion: We conclude that graft survival in CCD patients is shorter than in other patients with a non-inflammatory aetiology of CKD. However, despite this finding, kidney transplantation in CCD patients is still worth performing, as it improves survival rate and quality of life.