Endodermal sinus tumor is a malignant germ cell tumor. This rare tumor usually arise in the testis and ovary, although they have been discovered in such midline extragonadal sites as the retroperitoneum, sacrococcygeal area, anterior mediastinum, and central nervous system. These authors report a case of endodermal sinus tumor involving the ethmoid sinus, the nasal cavity and the retroorbital space with brief review of the literature.