Juvenile xanthogranuloma (JXG) is a common histiocytic disorder of infancy and early childhood, most frequently presenting with cutaneous lesions. Whereas involvement of several visceral sites is well documented, only 4 cases of primary testicular JXG have been reported. Although this benign disorder typically presents with a favorable clinical outcome, the unusual presentation as an intratesticular lesion can lead to diagnostic challenges. In this study, we present the case of a 6-month-old male child with an incidentally discovered testicular mass that was diagnosed as a JXG and briefly review the existing literature in an attempt to bring awareness to this uncommon presentation.
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