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Background: Short bowel syndrome (SBS) is a malabsorptive condition that develops as a result of massive resection of the small intestine and causes morbidities such as chronic diarrhea, dehydration attacks, parenteral nutrition (PN) dependence, and recurrent infections. Kidney transplantation in this patient group may be complicated by aforementioned morbidities, as well as the absorption problems of immunosuppressive drugs., Methods: We report the first pediatric patient (18-month-old male) with SBS secondary to volvulus who underwent a successful living related kidney transplantation with a primary diagnosis of autosomal recessive polycystic kidney disease and had a successful 4-year follow-up without intestinal transplantation., Results: Tacrolimus, mycophenolate mofetil (MMF), and prednisolone were administered for maintenance of immunosuppression after transplantation. The patient reached therapeutic trough levels of tacrolimus with usual doses. The 4-year renal survival was excellent without a clinical evidence of rejection, despite long-term necessity of PN and intravenous fluids., Conclusions: Kidney transplantation should not be avoided in patients with SBS solely because of concerns about the effectiveness of immunosuppressive therapy. Prednisolone, tacrolimus, and MMF combination was effective in our case, and these drugs can be considered as first-line agents in these patients., (© 2024 Wiley Periodicals LLC.)