This report describes a case of spontaneous ovarian hyperstimulation syndro
me (OHSS) occurring in a pregnant woman carrying the factor V Leiden mutati
on, Even though prophylactic treatment for thrombo-embolism was adopted by
administering low molecular weight heparin, the pregnancy was complicated b
y thromboses of the left subclavian, axillary, humeral and internal jugular
veins during the second trimester of gestation, The pregnancy was managed
conservatively and a healthy newborn was delivered at term. In order to avo
id unnecessary laparotomy, we emphasize the importance of careful diagnosis
in order to differentiate spontaneous OHSS from ovarian carcinoma, as well
as the necessity to look for the presence of coagulation disorders in wome
n affected by OHSS.