Erythroplasia of Queyrat (EQ) is a rare disease involving the mucosal and transitional surfaces of the penis. Effective treatment is necessary to minimize progression to squamous cell carcinoma. The standard therapy for EQ, partial or radical penectomy, is invasive; photodynamic therapy (PDT) may be an effective, non-surgical tissue-sparing option. We report the case of a 67-year-old patient with long-standing EQ who was suc-cessfully treated with methyl aminolevulinate-PDT (MAL-PDT). A complete clinical response, confirmedby incisional biopsy, was achieved af-ter fivesessions of every-other-week treatment. The patient experienced moderate edema, erythema and pain within 5-7 days after the treatment, without urination problems. Our experience and a review of the published literature suggest that MAL-PDT may represent a valuable treatment option for selected cases of histopathologically-confirmed EQ.