Exophthalmos is a common and important symptom in orbital consultation. It can be either uni- or bilateral. A wide spectrum of benign and malignant diseases has to be considered and evaluated for differential diagnosis, in order to maintain complete ocular function and to lead the patient to adequate therapy. Exophthalmos can be accompanied by variable symptoms, ranging from neurogenic or myogenic to corneal alterations. Symptoms at presentation depend on the underlying disease and may manifest systemically. Interdisciplinary teamwork is essential for diagnostics and therapy of exophthalmos. In addition to ophthalmological routine diagnostics, various supplementary examinations are available which are of importance for disease monitoring. Exact radiological imaging is important for the detailed visualisation of the pathology, surgery as well as treatment planning. Magnetic resonance imaging (MRI) and computed tomography (CT) are the standard imaging techniques used. Contrast enhancement and specific sequences can answer specific problems in detail. Combined positron emission tomography (PET) with CT permits evaluation of metabolic and morphological data and is employed in diagnosis of meningioma, lymphoma and metastases. In summary, the reader should learn important differential diagnoses and accompanying symptoms of exophthalmos, thus enabling essential clinical examinations and adequate imaging.