It has been recently reported that the occurrence of severe headache associated with temporary neurologic deficits and CSF lymphocytic pleocytosis is highly suggestive of the so-called “transient syndrome of headache with neurologic deficits and CSF lymphocytosis.” In particular, in almost all of the 40 patients reported in the literature to date, the head pain was severe and of a type not previously experienced by the patient. In the present case report, we describe a patient who fulfilled almost all the proposed diagnostic criteria, except for the lack of a severe headache. Probably, a severe headache is not a compulsory feature of this syndrome. Some patients have rather mild headache accompanying their episodes of neurologic symptoms, and some attacks occur without any accompanying headache. It is possible that in some cases the absence of a severe headache, and thus the lack of CSF analysis, lead to misdiagnosis. Therefore, the prevalence of this syndrome could be underestimated.