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The first case of KID syndrome in China was reported. A 21-year old female patient was diagnosed as KID syndrome in 2003 and followed up for 6 years till the time of writing. In 2008, an indurated nodule with mild exudates and adherent, keratitic scale developed on the back; 4 months later, another tumor developed on the left forearm. Both tumors were removed by surgical operation. Histopathologic diagnosis of the two tumors was squamous cell carcinoma and proliferating trichilemmal tumor, respectively.