Ewing’s sarcoma and peripheral primitive neuroectodermal tumour (pPNET) are now regarded as two morphological ends of a spectrum of neoplasms, characterised by a t(11;22) or other related chromosomal translocation involving the EWS gene on chromosome 22 and referred to as Ewing family of tumours (EFTs). They usually originate in bone or soft tissue but rarely arise in the vulva. Extra-skeletal Ewing sarcoma in pregnancy is rare. The current case report presents a case of PNET originating in the vulva in a 35-year-old pregnant woman. The patient benefited from a large excision of the tumor after giving birth to a healthy baby girl. She was treated for her pulmonary metastasis with conventional chemotherapy. She has had a stable disease with 6 months’ review. From this, physicians should give more attention to any vulva mass, especially in case of pregnancy that may accelerate the growth of Ewing’s sarcoma of the vulva, which can improve the prognosis of these pregnant women and their pregnancy.