Epstein-Barr virus (EBV) is a ubiquitous herpes virus whose infection is usually asymptomatic and persists for a lifetime. It often causes symptomatic diseases, such as infectious mononucleosis (IM), but rarely leads to EBV associated-NK-cell lymphoproliferative disorder (EBV-NK-LPD) in non-immunocompromised individuals. No studies have described EBV-NK-LPD treated with haploidentical hematopoietic stem cell transplantation (haplo-HSCT). The present study reports a case of a 29-year-old man with recurrent fever and cervical lymph node enlargement. The result of sorting lymphocytes with immunomagnetic beads was diagnosis of EBV-NK-LPD. To stop progression of this disease, haplo-HSCT was employed, with a good prognosis. The patient has survived for one year with no signs of recurrence.