Primary extragonadal germ cell tumours (PEGCT) are rare neoplasms with generally poor prognosis, different behavior, and natural course compared to their gonadal counterparts. Both primary and salvage treatment of these tumours constitute a challenge. Embryocarcinoma (EC) constitutes one of the subtypes of germ cell tumours. Its site of primary onset can either be gonadal or extragonadal, more frequent in infancy and childhood, the sacral and cranial regions being the most affected, while gonadal sites (ovary and testis) are more frequently involved in childhood. The authors observed a case of EC with nasal onset in a young male, never reported before in the literature. The patient underwent 6 courses of chemotherapy and further surgery by means of an endoscopic approach, without postsurgical sequelae. A 5-years follow-up, with periodic controls, laboratory tests and imaging, all without signs of recurrence, confirmed that this unusual location of EC responded exclusively to primary chemotherapy, while earlier studies proved EC being responsive, in other sites of onset, to a combination of chemotherapy, radical surgical excision of the neoplasm, and radiotherapy
