Sclerosing mucoepidermoid carcinoma of the salivary gland (SMEC) is a rare subtype of mucoepidermoid carcinoma (MEC), first described in 1987 by Chan and Saw. As far as we are aware, only 30 cases have been published since then. Most cases were located in the parotid gland with some cases described in the submandibular and minor salivary glands. SMEC typically presents as a long-standing mass, with a non-specific enhancing appearance on imaging and is often non-diagnostic on fine needle aspiration, making pre-operative diagnosis very difficult. It is characterised by dense sclerosis within an otherwise typical MEC, frequently with lymphoid proliferation and eosinophils at the periphery. The histological diagnosis of SMEC can be challenging, as the sclerosis may obscure the other morphological features, which can lead to misdiagnosis. Grading can also be difficult, and the prognostic value of grading for SMEC remains unclear. Herein is described a new case of SMEC, presenting clinically as chronic sialadenitis in the left submandibular gland of a 41 year old male. A brief literature review and the issues surrounding diagnosis and grading are also discussed.