von Willebrand disease is a genetic bleeding disorder that arises from abno
rmalities in von Willebrand factor, an adhesive glycoprotein involved in bo
th primary hemostasis and coagulation. It is the most common inherited blee
ding disorder in humans, and over the years several animal species have als
o been described as suffering from this disease whether through a spontaneo
us mutation (pigs, dogs) or a genetically engineered one (mouse). These dif
ferent animal models are extremely useful in exploring the characteristics
of von Willebrand disease and in testing new treatments. This review provid
es an update of the various von Willebrand disease models and the contribut
ion that these models can make to st better understanding of human von Will
ebrand disease.