Menarche is often when the disease first presents in women.

vWD is the most common inherited bleeding disorder, with a prevalence of about 1.3% in the pediatric population. It can be caused by either a qualitative or quantitative defect in von Willebrand factor, a protein that plays an important role in both platelet adhesion and the transportation of clotting factor VIII. vWD is inherited in an autosomal manner; there is marked heterogeneity in the extent of bleeding symptoms, given the variance in possible underlying protein defects. Even in families with identical mutations, the extent of bleeding may vary. Symptoms of vWD may include easy bruising, heavy postoperative or postpartum bleeding, gingival bleeding, bleeding following dental procedures or extractions, and menorrhagia (heavy cyclic menstrual bleeding).

Menorrhagia is reported in the majority of women with vWD and may in fact be the only manifestation of the disorder. Menorrhagia beginning at the time of menarche is typical, with one large study showing that 65% of women with vWD report menorrhagia beginning at menarche (vs. 9% of women with menorrhagia without vWD). Menorrhagia often affects quality of life around the time of menstruation, and can be severe enough to result in red blood cell transfusion or even hysterectomy or other measures to prevent severe bleeding. In addition to menorrhagia, women with vWD have a longer duration of menstrual bleeding per menstrual cycle. Adolescent girls may be particularly prone to menorrhagia; the concentration of von Willebrand factor increases with age, potentially decreasing menstrual bleeding over time.