Type 1 primary hyperoxaluria is an autosomal, recessive metabolic disease triggering calcium oxalate accumulation in tissues. The liver lacks the peroxisomal enzyme alanine-glyoxylate amino transferase. Clinically, patients develop kidney stones, urinary tract infections, and progressive renal failure. We evaluated liver and kidney transplantation to performed for type 1 primary hyperoxaluria in our study. In this study, we retrospectively evaluated 7 patients with type 1 primary hyperoxaluria who underwent liver and kidney transplantation. Two main groups were established; Sequential and simultaneous liver and kidney transplantation group. Mortality rates were compared between these two groups. Sequential liver and kidney transplantation was performed in 5 (71.4%) patients and simultaneous liver and kidney transplantation was performed in 2 (28.6%) patients. All patients died in simultaneous liver and kidney transplantation group. Sequential liver and kidney transplantation group mortality rates were lower therefore treatment for type 1 primary hyperoxaluria more preferable.