INTRODUCTION

Primitive neuroectodermal tumors (PNET) belong to the ewing sarcoma family of tumors. They are most prevalent in the second decade of life and are amongst the most common malignancies in the childhood and adolescent, compromising about 4% of the total malignancies[1, 2]. PNETs are a small round cell tumor with a neuroepithelial differentiation. Usually presenting as a bone of soft tissue mass in trunk or axial skeleton [3]. PNET of the adrenal gland is very rare. Up until 2019, 33 cases of adrenal gland PNET has been reported[4] with only 3 of them being retroperitoneal mases. Here we report a unique case of secretory PNET of the adrenal with cortisol secretion.