Hyperammonemia, post-orthotopic lung transplantation, is a rare but mostly fatal complication. Various therapies, including those to decrease ammonia generation, increase nitrogen excretion, and several dialytic methods for removing ammonia have been tried. We describe three lung transplant recipients who developed acute hyperammonemia early after transplantation. Two of the three patients survived after a multidisciplinary approach including discontinuation of drugs, which impair urea cycle, aggressive ammonia reduction with prolonged daily intermittent hemodialysis (HD), and overnight slow low-efficiency dialysis in conjunction with early weaning of steroids and other therapeutic measures. Our experience suggests that early initiation of dialysis, high dialysis dose, increased frequency, and HD preferably to less efficient modalities increases survival in these patients.