Primary hyperoxaluria is an inherited autosomal recessive disease leading to renal failure at various ages due to calcium oxalate deposition. Often, frequent stones at an early age are the first manifestation of the disease. Other tissues may be damaged from tissue deposits after renal failure develops. Therapy is directed at providing a normal glomerular filtration rate and oxalate excretion with a functioning graft, combined with measures to reduce oxalate production or precipitation in the urine and kidney. The disease may be a more frequent cause of renal failure than previously recognized and needs to be considered when the clinical findings are suggestive. Although there is an increased risk of recurrence of the disease in the transplanted kidney, with subsequent graft damage and loss, transplantation can be successful in a significant percentage of patients by using techniques that will optimize graft function and reduce the metabolic load of oxalate.