Although juvenile xanthogranulomas are frequently seen by pediatricians and dermatologists, urological data are extremely rare. To our knowledge we report the first case of juvenile xanthogranuloma of the penis. Differentiation from other, similar lesions with more ominous prognostic significance, such as xanthosarcoma, malignant fibrous histiocytoma or histiocytic reticulosarcoma, is essential. This well documented case is noteworthy, since proper ad hoc diagnosis would have allowed us to perform a small biopsy rather than 2 surgical procedures. Our data support the currently held belief that juvenile xanthogranulomas are generally benign, self-limiting lesions.