Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare group of chronic EBV infection-associated diseases, and usually occurs in childhood. The prognosis of HVLPD varies. Previous researches found that the prognosis of adults with HVLPD is worse than children. Herein, we report a case of HVLPD in an 8-year-old girl, but the clinical course progressed rapidly, from the classic form of HVLPD to severe HVLPD, and finally progressed into lymphoma. Fortunately, the patient was curable by hematopoietic stem cell transplantation. To the best of our knowledge, this is the most complete story of HVLPD in a same patient so far.