Primitive neuroectodermal tumor in adults is extremely rare. It belongs to the Ewing's sarcoma family that usually occurs in children and adolescents with a predilection for the truncal and axial soft tissue. Primitive neuroectodermal tumor of the posterior urethra is even less common with no prior reported cases in the English-language literature. We reported here a case of primitive neuroectodermal tumor of the posterior urethra with local bladder wall invasion and distant pulmonary metastasis in a 49-year-old man. The patient's treatment and clinical outcome were examined with literature review for the management of primitive neuroectodermal tumor in general with emphasis on its management in the genitourinary tract.