In this study, we report a case of hydroa vacciniforme lymphoproliferative disorder (HVLPD). The patient, who was 21 years old at the time of initial consultation，suffering from recurrent papules, vesicular rashes, bleeding and black scabs on the neck, face and trunk. Serum EBV-DNA was significantly increased (2.88×10⁷ copies/mL). The patient underwent skin biopsies twice within 2 years. The pathology of the first skin biopsy showed partial degeneration and loosening of the epidermal stratum spinosum, intraepidermal blister formation, partial epidermal detachment, and multifocal small abscesses seen in the blisters and stratum spinosum. Patchy infiltration of small lymphocytes, plasma cells, histiocytes, and eosinophils in the dermis, with no significant atypia of lymphocytes, EBER in situ hybridization was negative, which made it difficult to make a definitive diagnosis on pathology. The pathology of the second skin biopsy showed blisters visible within the patient's epidermis， and atypical lymphoid cells infiltrate around the hair follicles, sweat glands and blood vessels in the dermis. The immunohistochemical analysis indicated that lymphoid cells were positive for CD3, CD5, CD4, CD8, granzyme B and TIA-1, while CD56 and Perforin were negative, and the proliferation rate of Ki-67 was approximately 10%. EBER was positive by in situ hybridization consistent with clinicopathologic features of HVLPD. More than 1 year after receiving symptomatic treatment, the patient's rash worsened, with sometimes fever and left eyelid edema. The third skin biopsy performed in the other hospital showed that atypical lymphoid cells infiltrated the subcutaneous adipose tissue, and the proliferation rate of Ki-67 was 60%. The disease progressed to EBER-positive T-cell lymphoma. After 2 courses of chemotherapy with gemcitabine, cisplatin, dexamethasone and pegaspargase, the patient's edema subsided and the rash healed. This report demonstrates the clinical and pathologic features of the disease during its evolution and progression, with a view to enriching its diagnostic and therapeutic experience.