Abstract
•Invasive adenocarcinoma of the vulva arising from extramammary Paget's disease is possible in women of child-bearing age.•Radical vulvectomy, sentinel lymph node biopsy, and inguinal lymphadenectomy are safe and feasible during pregnancy.•Chemotherapy may be used as adjuvant therapy for vulvar adenocarcinoma during pregnancy when radiation is contraindicated.•Primary cesarean delivery may be considered for pregnant women with recent vulvar surgery.•Trastuzumab may be considered for maintenance therapy of Her2/Neu positive vulvar cancer in postpartum women.