Background: Sino-nasal teratocarcinosarcoma (SNTCS) is rare and unfamiliar entity with grave prognosis. It was first described by Shanmugaratnam et al. in 1983 and was aptly termed as __ampersandsignldquo;Teratocarcinosarcoma__ampersandsignrdquo; by Hefner and Hyams in 1984. As the name suggests, it is composed of benign and malignant epithelial components as well as mesenchymal and neural components Because of its rarity, these lesions are often misdiagnosed as immature teratoma or carcinosarcoma may lead to management difficulties. Objective: The objective is to make pathologists aware with this rare entity and always consider it as differential diagnosis of teratoma and carcinosarcoma which are more common lesions in this location / region. Research Methodology: We report a 30 years old female with SNTCS involving right nasal cavity extending into nasopharynx, maxillary, ethmoid, sphenoid and frontal sinuses. She presented with complains of the nasal blockage, difficulty in breathing and bleeding from right nose since 1 month. Excision biopsy was done. Result: Multiple sections have been taken and final diagnosis of Sino-nasal teratocarcinosarcoma was made.