Abstract
Primitive neuroectodermal tumors (PNET) are rare, small round cell tumors that are difficult to diagnose. It is important to identify PNET early, utilizing immunohistochemistry and genetic markers, as it is often an aggressive cancer. PNET is most commonly described in men between the ages of 20 and 40, with very few case reports highlighting the development in pregnant patients. We present a case of localized renal PNET in a pregnant patient and highlight the diagnostic work-up and treatment as well as the relationship between pregnancy and the potential development of aggressive tumors.