Paraneoplastic neurological syndrome (PNS) includes rare manifestations of different forms of cancer, in which the specific syndrome of limbic encephalitis can be found. This report describes a case of a previously healthy young lady who developed severe limbic encephalitis associated with an immature teratoma. After surgical treatment, the patient showed rapid progressive neurological improvement with complete regression of the symptoms during follow-up. Although rare, correct recognition and management of PNS is of great importance especially considering the fact that PNS can carry the risk of permanent disability or even death,even when associated with tumors in which high cure rates are expected.