Rare causes of headache syndromes could be easily misdiagnosed as common causes of headaches like migraine, episodic tension-type headache, or trigeminal neuralgia. Sometimes, that can be hard to diagnose. The time for diagnosis and treatment can be delayed by months or even years. Hence, some of these headache syndromes are associated with structural and vascular abnormalities of the brain; it is essential to recognize this type of syndrome rapidly. In this chapter, we report on a 67-year-old male who has been searching for a remedy to relieve his headaches for three years. The patient has unilateral persistent daily headache accompanied by autonomic symptoms. The patient’s history and lack of response to indomethacin and oxygen tests suggest the diagnosis of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). When SUNCT is diagnosed, imaging should be performed to rule out secondary causes. Depending on the frequency and severity of the patient’s pain, acute lidocaine infusion therapy or prophylaxis with lamotrigine may be started. In cases where lamotrigine is not sufficient, protection can be provided with additional medication. Surgical procedures may be performed in refractory patients.