Wilms tumour (WT) (or nephroblastoma) is one of the most common malignant kidney tumors in children. On subsequent stages clinically it is often characterized by abdominal hypertension syndrome, which, in turn, leads to development of respiratory insufficiency. Other symptoms comprise renal deficiency, hypertension, and abnormalities of hemostasis and hemogram. Treatment includes rounds of preoperative chemotherapy and subsequent surgery. We report a case of perioperative management for nephrectomy in 20-month-old patient with a giant unilateral WT. The complexity of anesthesia was determined by the size of tumor, increased intra-abdominal pressure, respiratory deficiency, and hypercoagulation.