Abstract
•Vulvar yolk sac tumor (YST) is an exceptionally rare and aggressive extra-gonadal germ cell tumor, previously only described in 15 cases in the literature.•We present a unique case of vulvar YST diagnosed during pregnancy, treated with systemic chemotherapy, who subsequently recurred during a second pregnancy.•Despite salvage chemotherapy and autologous stem cell transplantation at recurrence, our patient succumbed to her disease, 31 months post diagnosis.