Many patients who present with otalgia have a normal otological examination, and a distant source of pain must be considered. The ear receives an extensive sensory innervation arising from six nerve roots. Many other structures in the head, neck and thorax share a common neuronal pathway with the ear, and these tissues represent the possible sites of disease in the cases of referred otalgia. Consequently, the differential diagnosis is extensive and varied. Making an accurate diagnosis relies on an understanding of the complex distribution of nerve fibres and a structured approach to patient assessment. This article aims to classify the aetiology of referred otalgia and to outline current treatments for these conditions. The origins of referred otalgia may be as remote as the cranial cavity and thorax; however, dental disease, tonsillitis, temporomandibular joint disorders and cervical spine pathology represent the most frequent causes. Ear pain may also be the first sign of a head and neck malignancy. Patients complaining of otalgia, with risk factors for an aerodigestive neoplasm, and a normal ENT examination require an urgent otolaryngological opinion.