Headaches are among the most common complaints in the Neurology Clinic. As a symptom, headache is often a problem not only for the patient, but also for the doctor, as in addition to primary headaches, secondary headaches can have various etiologies. Among the primary headaches, migraine and cluster headaches may present cranial nerve symptoms. The differential diagnosis of secondary headaches can be difficult. We have to include vascular, neoplastic, infectious, inflammatory, toxic, autoimmune causes, where diagnosis requires complex laboratory and/or imaging studies, while the absence of abnormal findings often suggests autoimmune or unknown origin. In our review, we aim to present three cases where the headache was associated with cranial nerve involvement, mainly ophthalmoparesis. The course of the disease was long in all three cases, characterized by symptom-free periods alternating with paroxysmal headaches and diverse cranial nerve palsies. Most frequently, the oculomotor and abducent cranial nerve palsies were observed, but the involvement of trigeminal and facial nerves occurred as well. Based on the symptoms, there was a suspicion of a lesion of the cavernous sinus in all three cases, however, this couldn’t be confirmed by advanced neuroimaging techniques. In addition to symptomatic treatment, steroid therapy improved the complaints, but neurological signs did not disappear in all cases. Furthermore, the signs reappeared after a short time or a different cranial nerve was involved. The lack of a positive diagnosis, the fluctuating course of the disease, and the side effects of steroid therapy demand a long-term follow-up, which is possible only through an appropriate doctor-patient relationship. As guidelines are difficult to elaborate on for such cases, continuous re-evaluation of diagnosis and treatment are required.