Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily observed during the first 2 years of life. Most patients present with a solitary cutaneous lesion; however, others present with extracutaneous manifestations or even with systemic involvement. The authors describe a 2-month-old boy in whom was diagnosed a unifocal extracutaneous JXG involving the temporal bone. Unlike 3 other cases of solitary JXGs of the temporal bone in the literature, the present case involved destruction of the dura mater and leptomeningeal enhancement surrounding the entire temporal lobe. The lesion did not regress after an initial biopsy procedure and had to be removed more radically because of progressive mass effect on the brain. The child recently underwent a reconstructive skull procedure and is doing well almost 2 years postoperatively without evidence of disease. This case demonstrates that even in instances of extensive disease a favorable outcome is possible without chemotherapy.