<p>Crimean-Congo hemorrhagic fever is a severe tick-borne viral infection with high mortality rates. While Crimean-Congo hemorrhagic fever primarily presents as a hemorrhagic fever, central nervous system involvement, including encephalitis, is rare. The virus, transmitted through tick bites or direct contact with infected animal blood or bodily fluids, can lead to multi-organ failure. Neurological manifestations of Crimean-Congo hemorrhagic fever remain poorly understood. We report a 40-year-old man from Hormozgan province, Iran, who presented with fever, hematemesis, abdominal pain, and neurological symptoms. Initial laboratory findings indicated thrombocytopenia and elevated liver enzymes. Despite treatment with ribavirin, the patient developed agitation, confusion, and a progressive decline in consciousness. Brain imaging suggested encephalitis, and cerebrospinal fluid analysis revealed mild pleocytosis with elevated protein levels. Crimean-Congo hemorrhagic fever was confirmed via polymerase chain reaction testing. The patient was treated with ribavirin, intravenous immunoglobulin, and high-dose methylprednisolone, gradually recovering neurological function. Crimean-Congo hemorrhagic fever with encephalitis is an uncommon but severe presentation, necessitating prompt diagnosis and intervention. This case highlights the potential role of corticosteroids and intravenous immunoglobulin in managing Crimean-Congo hemorrhagic fever-associated neurological manifestations. Further studies are needed to establish standardized treatment protocols for Crimean-Congo hemorrhagic fever-related encephalitis.</p>