Juvenile xanthogranuloma (JXG) is a benign proliferative disorder of dermal dendrocytes which usually presents with skin lesions. Renal involvement is extremely rare. We present a 40 day old female infant. Radiologic imaging techniques revealed a renal mass, prompting a preliminary diagnosis of a renal tumor. Consequently, nephrectomy and histopathological examinations were performed yielding a diagnosis of “JXG”. The patient did not exhibit any pathognomonic skin lesions. This case is unique with its unusual clinical presentation. Although renal involvement of JXG is very rare, it should be kept in mind in clinical differential diagnosis in infants and children presenting with renal mass.