Out of 99 total HLH patients, fourteen (median age 48 years, range 21-71) were identified as having neurologic symptoms attributable to HLH.  Eight (57%) had neurologic symptoms at disease onset. The majority (64%) presented with encephalopathy but one presented with hemiatixia and one with diplopia. Three patients (21%) had prominent peripheral nervous system involvement. Clinical course was complicated by seizures in three patients, and stroke in two (one ischemic, one hemorrhagic). In most patients, HLH was associated with either malignancy (36%), or infection (36%). Three (21.5%) had no identifiable associated systemic condition.  Of the 9/14 patients who underwent lumbar puncture, the majority (89%) had elevated protein but only three (33%) had pleocytosis. Brain MRI was done in twelve patients with a wide variety of findings. Seven patients were treated with etoposide and dexamethasone, and in six, treatment was directed at the underlying condition. Only two patients were alive at the time of our review.