The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare entity. This disease has been related with migrainous headaches. It is a benign disorder, self-limited, and characterized by fluctuating neurological symptoms and cerebrospinal fluid (CSF) lymphocytosis. A small proportion of patients develop intracranial hypertension as a consequence of the illness. Confusional state may be also observed.We describe a case of a 29 years old man with acute onset of headache, dysthermia sensation, vomits, aphasia, and difficulty to perform daily activities such as unlocking the mobile phone. Blood tests as well as computed tomography (CT) and magnetic resonance imaging (MRI) were normal. Cerebroespinal fluid analysis revealed a lymphocytic pleocytosis (47 cells/µl, 100% lymphocytes). Electroencephalogram showed moderate slow rhythm in the left hemisphere, with frontotemporal predominance, and without epileptiform activity. With the diagnosis of HaNDL syndrome the patient was accepted in the Department of Neurology and discharged asymptomatic without treatment.