OBJECTIVE To update the clinical characteristics of mycosis fungoides in Chinese patients. METHODS We retrospectively analyzed the clinical data of 51 patients (29 men and 22 women) with mycosis fungoides in PUMC hospital from 1984 to 2006, to determine the ages at diagnosis, clinicopathologic characteristics of skin lesions, systemic manifestation, misdiagnosis and treatment of these patients. RESULTS The mean age was (44.24 +/- 2.05) years at the diagnosis. Most patients were characterized by the typical evolution of patches, plaques and tumors, with some variations and subtypes. Clinical manifestations included generalized lesions (68.6%) and itchy (70.6%). Epidermotropism (68.6%) and Pautrier's microabscesses (52.9%) were common histopathologic features. The positive rate of T-cell receptor gene rearrangement was 81.3%, and was independent of the histological features. Previous misdiagnosis rate was 64.7%. Skin-targeted therapies and biologic therapies were effective approaches to relieve the skin rash at early stage, and combined chemotherapy was typically applied in more advanced cases. CONCLUSIONS Mycosis fungoides has various clinical characteristics and careful differential diagnosis should be made in clinical practice.