Thrombotic events in patients with inherited bleeding disorders occur only rarely. However, in some cases, the co-existence of acquired or inherited prothrombotic risk factors may overcome the hypocoagulative state, modulating the clinical phenotype to a decrease in bleeding symptoms or even to an increase in the likelihood of developing thrombotic complications. This review summarizes the cases of thrombosis reported in the literature and analyzes the most important risk factors for thrombosis in patients with a congenital bleeding tendency. Data were identified by searches of the published literature, including PubMed, references from reviews and abstracts from the most important meetings on this topic. There is increasing evidence that thrombotic complications in patients with hereditary bleeding disorders have a multifactorial pathogenesis, depending on acquired (coagulation factor replacement therapy, central venous catheters, surgery, viral infections) and/or inherited (thrombophilic gene mutations) prothrombotic risk factors.