Introduction: Pleomorphic adenomas are the most common non-malignant neoplasms of salivary gland tumours that present mixed histopathological patterns. The purpose of our study was to report an unusual case of a pleomorphic adenoma which was located in the upper lip that had been initially considered as cyst. Case report: The patient was a middle aged man who had a painless, slow-growing swelling in the upper lip. At first, the round-shaped and mobile mass was considered as cystic lesion. However during the operation, it was discovered instead of a cyst, an encapsulated solid tumour that demonstrated both epithelial and mesenchymal cells in histopathology and the diagnosis of pleomorphic adenoma was set. One year postoperatively the patient had no recurrence of tumour. Conclusions: Occurrence of intraoral lip swelling could be attributed, even rarely, to benign mixed tumours. Despite its nonmalignant features, these masses have a relatively low but still existing risk for recurrence or malignant transformation and thus in similar cases a systematic follow-up should be performed even years after surgery.