Six years old male child known case of β – Thalessemia Major from 6 months of age, who is on repeated blood transfusions admitted with failure to thrive, breathlessness and reduced activity. Complete investigation was done for this child who showed microcytic hypochromic anemia, thrombocytopenia, prolonged bleeding and clotting time, abnormal liver function test and Tricuspid regurgitation grade III with moderate pulmonary hypertension and cardiomegaly. With the above high risk complications the child was posted for elective splenectomy and managed successfully.