OBJECTIVE: Basal cell adenocarcinoma of a minor salivary gland is extremely rare. The goal of this report is to increase awareness of this rare disease and to review and discuss the differential diagnosis and important considerations in treatment. STUDY DESIGN: Case report and review of the literature. METHODS: Case report of a basal cell adenocarcinoma of a hard palate minor salivary gland and review of the literature of basal cell adenocarcinoma. RESULTS: Basal cell adenocarcinomas are slow-growing tumours that most commonly involve the parotid gland and very rarely involve minor salivary glands. Although recurrence rates for these tumours are high, mortality rates are low. Histological diagnosis is important to distinguish this tumour from adenoid cystic carcinoma given the significant difference in disease prognosis. CONCLUSIONS: Diagnosis of these tumours must be made histologically. Recommended treatment options include wide local excision with radiotherapy reserved for close surgical margins or for local recurrence.