All reported cases of primary squamous cell carcinoma of the endometrium and the effectiveness of the different treatment modalities in the world literature are reviewed. We present the first reported case of primary squamous cell carcinoma of the endometrium treated by radical hysterectomy, pelvic and para-aortic lymphadenectomy. The patient is without signs of disease 4 years after surgery. This is the longest follow-up of all reported cases. There are very few cases from which is to conclusively predict the best mode of therapy. The current standard of treatment is total abdominal hysterectomy and bilateral salpingo-oophorectomy with or without pelvic and para-aortic lymph node sampling, depending on the histology and depth of the myometrial invasion. Adjuvant therapy includes radiation therapy according to the extent of disease.