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 indurat-ed nodule with mild exudates and adherent, kemtitic scale developed on the back; 4 months later, another tu-mor developed on the left forearm. Both tumors were removed by surgical operation. Histopathologic diagno-sis of the two tumors was squamous cell carcinoma and proliferating trichilemmal tumor, respectively. Key words: KID syndrome; Neoplasms,squamous cell; Proliferating trichilemmal tumor