Kimura disease is a rare, benign, chronic disease of unknown etiology. It is characterized by recurrent tumor-like lesions in the soft tissues localized mainly in the head and neck region with peripheral eosinophilia, and elevated serum total IgE levels. The diagnosis is confirmed by histopathological examination. Kimura disease may be associated with glomerulonephritis. This disease usually occurs in men, mainly of Asian origin in the second and third decade of life. In this report, the authors present a case of 15-year-old boy treated for nephrotic syndrome. During hospitalization, peripheral lymphadenopathy was observed in the pericubital area of both forearms. It was diagnosed for the first time in June 2010 and in December 2010, the histopathology defined it as reactive lymphadenopathy. Considering the late onset of the nephrotic syndrome after 12th year of life with the simultaneously present peripheral lymphadenopathy the decision of repeated oncological screening was made to exclude neoplastic transformation. In the second lymph node histopathological examination the characteristic picture for Kimura disease was obtained. The authors emphasize very rarely occurring diseases, atypical for the local population and the age of onset, may create difficulties both for diagnostic and therapeutic procedures in pediatric practice.