a 73 year - old patient with mild hemophilia b ( factor ix clotting activity  12% ) was referred to our department due to a left renal tumor incidentally discovered during ultrasonography performed as follow - up two years after prostate cancer brachytherapy . on admission abdominal and pelvic ct scans revealed a multilocular tumor in the lower left kidney pole ( 57x52x52 mm ) with a solid mass of 3.5 cm in diameter ( figure 1 ) . other abdominal and chest imaging showed no abnormalities . twelve hours before surgery , he was transfused with 4200 units of factor ix concentrate ( octanine , octapharma ag , switzerland ) , with the same dose administered one hour prior to the procedure . on october 3 of 2014 , the procedure was performed with the patient positioned on his right side . following urinary catheterization , a veress needle was introduced close to the umbilicus and an intraperitoneal pressure of 14 mmhg was created . the descending colon was dissected and the fat renal capsule subsequently cut for access to the left renal artery . the artery was then clipped with polymeric clips ( hem - o - lok xl ( teleflex , usa ) and cut in between . the same was done for the renal vein . the kidney was excised , placed into a hemobag ( covidien , usa ) and removed through a small incision between the two ports . a plastic drain was placed into the kidney bed and the abdominal wall was closed with layers of sutures . peritoneal drainage was kept in place for two days with no significant output of blood or serous fluid . substitution therapy was continued for 9 days with the level of factor ix clotting activity at 90130% of the normal value on days 13 , 6574% on days 46 and 3040% on days 79 . the postoperative course was uneventful and the patient was discharged on 10 day after surgery . histopathological investigation revealed renal carcinoma , clear cell type ( g2 ) , limited to the parenchyma , without capsule infiltration or angioinvasion . no abnormalities were revealed in ultrasonography and ct scans . computed tomography scan revealed a left kidney tumor mass . hemophilia b ( christmas disease ) is a congenital bleeding disorder resulting from factor ix deficiency . hemophilia b patients undergoing surgical procedures are at a high risk of severe bleeding complications unless surgery is performed after adequate correction of the diathesis . secure hemostasis in hemophilia b patients is achieved with transfusions of factor ix coagulation factor concentrate , either recombinant or plasma - derived . desirable preoperative activity is 6080% of the normal value and should be maintained until complete wound healing , usually through 710 days . on days 13 the recommended value is 6080% , on days 46 4060% and 3040% on days 710 . minimally invasive procedures in patients with hemophilia , apart from well - known benefits such as reduced postoperative pain , fewer complications , better cosmetic effect , shorter hospitalization , quicker convalescence and return to professional activity , also contribute to shorter duration of substitution therapy . the first laparoscopic nephrectomy was performed by clayman et al . in 1990 . since then , the procedure has been performed in an increasing number of urologic centers [ 4 , 5 ] . there are only few reports in the literature of urinary tract operations in patients with congenital coagulation disorders [ 6 , 7 ] ; nonetheless , there have been none of laparoscopic nephrectomy in a hemophilia patient . we present the first case of laparoscopic nephrectomy in a hemophiliac performed with an adrenal - saving , transperitoneal lateral approach using four trocars . no significant complications were observed either postoperatively or in the long - term , which can most likely be attributed to the adequate preparation of the hemophilia patient for surgery , as well as the effective cooperation between urologist and hematologist . perioperative and postoperative bleeding episodes are the most frequent complications reported in hemophilia patients . for patients with no coagulation disorders , tlrn intraoperative bleeds are observed at 2.22.8% , and are frequently a reason for conversion to an open procedure . there are no significant differences in the reported percentage of all surgical complications , hemorrhage included , between hemophilia patients with adequate substitution of deficient coagulation factor and patients with no coagulation disorders [ 8 , 9 ] . it is therefore crucial for hemophilia patients to undergo surgery in special reference centers , which provide expert health - care services and are equipped with a modern laboratory for careful daily monitoring of deficient coagulation factor activity [ 9 , 10 ] . in conclusion , laparoscopic radical nephrectomy in hemophilia patients is a safe and effective procedure for renal carcinoma . surgery should be performed by an experienced urologist in cooperation with a multidisciplinary team , providing both perioperative and postoperative care , supported by a specialized laboratory .