BACKGROUND AND PURPOSE Although reports of single cases of painful ophthalmoplegia (PO) are common, studies considering larger case series are lacking. Here, we aimed to determine the relative frequencies of ocular neuropathies, the causes, the usefulness of diagnostic procedures and the role of steroid treatment in PO. METHODS Between January 2006 and September 2012, 149 patients' charts who presented with diplopia in our emergency department were studied retrospectively. 34 of them met the inclusion criteria that included recent (≤3 days) symptom onset and a minimum of diagnostic work. RESULTS 32% of single or combined ocular motor nerve palsies were of diabetic microvascular etiology and most of them were IIIrd or VIth nerve neuropathies. The most useful, in terms of sensitivity and specificity of diagnostic test in the acute setting was ESR, whereas MR-angiography and focused cavernous sinus imaging led to diagnosis in the post-acute phase. Pain response to steroids was non-specific, in contrast to palsy improvement after steroid administration which was indicative of Tolosa-Hunt syndrome or temporal arteritis. CONCLUSIONS Although acute and subacute PO might be intuitively associated with Tolosa-Hunt syndrome or sinister pathology such as aneurysmal hemorrhage, our data show that these causes are far less common than diabetic microvascular palsies. Brain CT, MR-imaging of brainstem, cerebellum or hemispheres, CSF analysis and pain response to steroids are nonspecific and hence less helpful in order to arrive at a diagnosis. Instead, improved ocular motility after steroid treatment, as well as MR-angiography and cavernous sinus imaging appear more useful for this purpose.