Background Medullary sponge kidney (MSK) disease predisposes patients to recurrent nephrolithiasis, which affects one in every 5000 people in the United States. Methods We report a rare case of a pediatric recipient of a living donor MSK transplant and discuss considerations when discussing risks and benefits of accepting MSK allografts for this population. Results The recipient was admitted due to concerns for nephrolithiasis, hydronephrosis, and urinary tract infection at 1‐month post‐transplant. The hydronephrosis was resolved by surgical removal of an encrusted ureteral stent; this was followed by supplementation with oral medications to prevent future episodes of nephrolithiasis. The recipient did not have any further episodes after this as seen at a 1‐year follow‐up. The donor has remained well through this period. Conclusions With increasing organ shortages, the use of variety of donors may need to be considered to enlarge the organ pool.