Teratomas affecting the head and neck are rare tumours of ambiguous origin. The benign form is seen in children and mostly affects the nasopharynx. Malignant tumours in the upper jaw have been reported rarely. They are almost exclusively seen in the adult male. They are highly aggressive tumours that are difficult to diagnose pre-operatively due to the varied histological patterns. Treatment options available are surgery, radiotherapy or a combination of both. Despite intensive treatment, the prognosis is still poor. A rare case of teratocarcinosarcoma of the nose, paranasal sinuses and nasopharynx in a 25-year-old male is presented. The tumour had a non-germ cell tumour origin. The clinical features, pathologic characteristics and treatment are detailed. It was managed by surgery, radiotherapy and chemotherapy.
