Atypical haemolytic uremic syndrome is a disease caused by complement regulation abnormalities that generally progresses to chronic end-stage renal disease with a high rate of recurrence in kidney transplantation and a high risk of graft loss. Anti-complement therapy has improved the prognosis of these patients, achieving disease remission in most cases, increasing the likelihood of a successful kidney transplant and increasing patient and graft survival. Drugs with low risk of induction of thrombotic microangiopathies such as belatacept and mycophenolate have also been used with satisfactory results. We present the case of a young patient at high immunological risk, with atypical haemolytic uraemic syndrome due to factor H mutation, who underwent a successful kidney transplantation with eculizumab, thymoglobulin, belatacept, mycophenolate and steroids, to date preserving excellent graft function without disease recurrence.