This report describes the perioperative management of disseminated intravascular coagulation occurring abruptly during a planned cystectomy for non-metastatic bladder papillary carcinoma. Peroperatively, profuse bleeding and an acute decrease in blood pressure were effectively treated by blood transfusions and fresh frozen plasma. Haematological tests indicated the presence of disseminated intravascular coagulation. On the following three days, acute renal failure, peripheral disseminated intravascular coagulation-related thrombocytopenia and haemolytic anemia with schistocytes were suggestive of thrombotic microangiopathy. Treatment by plasma exchange along with haemodialysis was commenced. An aetiological work-up remained negative. After 21 days of treatment, haemodialysis and plasma exchange were stopped. Urological outcome was favourable. The one-year follow-up did not show any residual renal insufficiency and laboratory parameters returned to normal. In the absence of evidence in favour of an infectious, drug-related or immunological aetiology, we postulated that this thrombotic microangiopathy was caused by disseminated intravascular coagulation and that the tumour manipulation during the surgical procedure was the triggering factor.