Malignant lympho-epithelial lesion of the salivary gland is an undifferentiated squamous cell carcinoma which is associated with a prominent lymphocyte-rich stroma. This study reviews the current literature pertaining to this rare tumour with reference to the single recorded case of malignant lympho-epithelial lesion of the salivary gland presenting in Auckland, New Zealand. This 26 year old Caucasian male developed a rapidly growing lesion in the right parotid gland and multiple ipsilateral cervical lymph nodes. Treatment consisted of surgery and radiotherapy. The patients is currently disease free. One hundred and eight other cases have been reported in the literature, 82 of whom have Mongolian ancestry (Eskimos and southern Chinese) and 15 of whom are Caucasian. More than 50% of these patients (all Mongolian) have elevated titres of serum immunoglobulin A against Epstein-Barr virus capsid antigen. The age at presentation is variable, ranging from 10 to 86 years, with a slight female predominance. Forty-one per cent of patients present with regional lymph node metastases and 20% with distant metastases. Given the rarity of this disease, treatment is controversial. Current data suggest that early radical combined modality treatment using surgery and radiotherapy leads to prolonged survival and cure with 5 year survival rates greater than 50%.