Abrupt headaches with focal neurologic deficits usually indicate medical emergencies that require advanced investigations and prompt treatment. The distinct syndrome of “headache with neurologic deficits and cerebrospinal fluid lymphocytosis” (HaNDL) is infrequent and considered a benign, self-limited disorder. We illustrated a 29-year-old man with HaNDL atypically presenting as episodic altered consciousness rather than sensorimotor prodromes. The diagnosis was made carefully by exclusion of other likely entities. It is important for clinical physicians to differentiate HaNDL from other potentially fatal.