Objective To investigate clinical manifestations,pathology,surgical treatment and prognosis of oculocutaneous malignancy secondary to xeroderma pigmentosum.Methods This was a retrospective case series study.The case records of 5 malignancy patients secondary to xeroderma pigmentosum visited Beijing Tongren Eye Center between 1999 and 2011 were reviewed,and the mean age of the patients was 44.6±4.9 years.Surgical and other treatment and immunohistochemical staining were analyzed.Results The results of pathological and immunohistochemical examinations showed that 1 case had malignant neurinoma,and 3 cases had basal cell carcinoma,and 1 case had malignant melanocytoma.All these cases were followed-up for 3 to 6 years.In 2 cases the malignancy relapsed,one was squamous cell carcinoma,another trichoepithelioma.Conclusion Oculocutaneous tumor secondary to xeroderma pigmentosum usually is malignant,and tends to recur.Although the characteristics of this disease include a rapidly progressive clinical course of the malignant tumor in the eye and a poor prognosis,timely surgical removal will significantly improve the prognosis.Massive resction and skin grafting should be performed during the surgery according to the results of pathological examination on the cryostat sections,and the range of surgical treatment should be large enough.