Pseudoepitheliomatous hyperplasia (PEH) is down-growth proliferation of the epidermis and of the adnexal structures into the dermis, mimicking a well-differentiated squamous cell carcinoma. It might be associated with many different conditions, mostly benign but rarely with some malignant conditions. Association of PEH with cutaneous lymphomas has occasionally been reported in the literature. Here, we report a 70-year-old man who presented with vegetative nodules clinically appearing as a tumor stage of mycosis fungoides,besides typical plaques of the disease. Histopathological examination of the tumor lesion revealed that the bulk of the tumor was due to PEH. During the follow-up period lymph node involvement was determined. We considered that mycosis fungoides cases associated with PEH should be followed up closely.