Venous and arterial thrombosis due to a constitutional protein S defic
iency is well-known. We report the case of a 36 year-old patient admit
ted to hospital in 1991 for primary renal vein thrombosis due to a con
stitutional protein S deficiency of type I. The diagnosis was made by
CT scan and angiography. Left nephrectomy, which was made because of d
oubt with regard to subjacent neoplasm, showed left renal vein thrombo
sis and multiple renal infarcts. In 1994, after 4 months of discontinu
ation of oral anticoagulants, the patients presented pulmonary embolis
m documented by pulmonary scintigraphy and CT scan, partial portal thr
ombosis and sural thrombophlebitis documented by echography coupled wi
th Doppler. To our knowledge, this is the first reported case of a con
stitutional protein S deficiency associated with primary renal vein th
rombosis.