ABSTRACTvon Willebrand disease (vWD) is the most common type of autosomally inherited bleeding disorder affecting up to 1% of the world's population. The disease represents a range of quantitative and qualitative pathologies of the adhesive glycoprotein, von Willebrand factor (vWF). Since symptoms are often mild, a significant majority of patients remain undiagnosed. In the hemostasis laboratory, the measurement of vWF antigen and ristocetin cofactor activity is key components in the diagnostic algorithm for vWD. With all forms of vWD, however, bleeding episodes can be severe and may require treatment, particularly during or following surgery or dental work. The primary care physician can and should play a role in recognizing the signs and symptoms of vWD and in referring patients for proper management. The treatment of bleeding in vWD involves the use of desmopressin and plasma-derived vWF concentrates and a variety of adjunctive agents. This article gives a detailed description of the case report on a 5-year-old boy who presented with vWD in dental clinic and also imparts the knowledge on back ground, pathophysiology, classification, diagnostic measures and treatment modalities of this fatal bleeding disorder from dental perspective.