Kimura disease is a chronic inflammatory disease,which frequently affects middle-aged Asian men, although children are seldom affected by it. Therefore, the characteristics of the Kimura disease of childhood have not been well illustrated. In this report, we have described a case of Kimura disease and have summarized 29 childhood cases reported in Chinese people from 1988 to 2009 by using 3 Chinese journal search engines. Most of these cases were from provinces near the eastern and southern coast of China. Boys were predominantly affected. Head and neck are the most frequently involved sites of subcutaneous masses (24 of the 29, 82.8%). On laboratory investigations, peripheral blood eosinophilia (25 of 26, 96.2%) and elevated immunoglobulin E level (13 of 13, 100%) were common. Kimura disease was associated with high incidence of nephritic syndrome (8 of the 29, 27.6%) and eczema rash (13 of 29, 44.8%). Surgery excision, steroids, and chemotherapy were the main therapy methods in this cohort. For patients with follow-up data, 12 experienced recurrence, with a recurrence rate of 60.0%. Pediatricians need to be aware of this disease when dealing with patients with lymphadenopathy. A standard and effective treatment protocol would improve the outcome of the Kimura disease.