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Varicose veins in the vulvar and perivulvar area are seen in 4% of women. Most of them are secondary to pregnancy and usually regress spontaneously. Vulvar varicose veins are rare in nonpregnant women. When present, they can be seen alone, associated with leg varices or associated with venous malformations of the labia, clitoral area, or vagina with or without arteriovenous malformations on the limbs or trunk (Klippel-Trenaunay-Weber syndrome and Parkes-Weber syndrome). In some cases, vulvar varices are seen as part of the so-called "pelvic congestion syndrome." Clinically, vulvar varices may present as small isolated protrusions, mainly in the labia majora, or as large masses, involving the vulva and even the perivulvar area. The treatment of choice of vulvar varices seen during pregnancy is conservative and symptomatic. Surgical pathologists need to be aware of the existence of vulvar varicose veins and its possible presence in biopsy specimens. Vulvar varicose veins can be misdiagnosed clinically as cysts or masses mainly in the Bartholin gland area. Correct diagnosis of the lesion is important to determine appropriate therapy and to recognize the possibility of associated anatomical or pathological problems.