Painful ophthalmoplegia is a rare pathologic condition caused by non-specific inflammation of the cavernous sinus, but other causes such as tumours, vasculitis, basal meningitis, neurosarcoidosis, diabetes can be responsible for the syndrome. Aim of this study is a review of the cases of painful ophthalmoplegia admitted to our Institute in the last 20 years in order to verify the incidence of symptomatic versus benign forms in a clinical case series, with particular focus on the cases in which a long term (at least 4 years) and detailed follow-up did not revealed spread of any systemic disease or other presumed causes for painful ophthalmoplegia. Twenty-three patients were retrospectively studied, 12 patients (52%) were classified as benign forms and their disease course was again evaluated and 11 cases (48%) were designated as symptomatic. The present study suggests that in the clinical practice the incidence of benign forms among the painful ophthalmoplegias is more elevate than the symptomatic ones and underlines the need of a specific nosography for benign forms.