Primitive neuroectodermal tumor (PNET) most often presents as a bone or soft tissue mass in the trunk or axial skeleton in adolescents and young adults.1 It is highly aggressive and rarely arises in the kidney.2 A combined therapy, consisting of surgical resection,chemotherapy and radiotherapy, is needed to treat this tumor but long-term survival remains poor.3,4 In the current study, we describe a case of 26-year-old woman (primigravida) who presented with a PNET of the right kidney with inferior vena cava (IVC) tumor thrombus.