Menorrhagia is a common health problem in women, particularly those with bleeding disorders. Little is known about the course of menorrhagia or other bleeding symptoms in women with the most common congenital bleeding disorder, von Willebrand disease (vWD). We determined the prevalence of menorrhagia, bleeding symptoms and coagulation abnormalities associated with vWD, including factor VIII activity, von Willebrand factor (vWF) antigen, ristocetin cofactor and bleeding time (BT), on a cohort of 38 females with type 1 vWD referred for diagnosis and medical care. Menorrhagia was the most common bleeding symptom in females with vWD, occurring in 93.1% of adult women. Menorrhagia was also the most common initial bleeding symptom, occurring in 53.1% of adult women in all of whom it began at menarche, median 14 years of age. There was a delay from initial bleeding symptoms, at median age 12 years, to diagnosis, at median age 16 years, P=0.0049. Although 94% undergoing surgery had previous bleeding, a vWD diagnosis was known preoperatively in only 6.2%, resulting in potentially preventable bleeding. In summary, menorrhagia is the most common bleeding symptom in females with vWD and begins at menarche. Obtaining a personal and family bleeding history promotes early diagnosis, potentially prevents postoperative bleeding, and improves the health of women with vWD.