Juvenile xanthogranuloma (JXG) is an uncommon histiocytic cutaneous lesion. It is a type of non-Langerhans cell histiocytosis(WHO Class IIb). The mean age of onset is 2 years of age. The adult form of JXG is relatively rare. The most common affectedarea is the face or the scalp and most of the lesions are less than 5 mm in diameter. This lesion tends to show a self-limited course over several months to years. However, large size JXG can have an atypical course or create cosmetic problems. Therefore,excision is considered in such lesions. We report an adult form of JXG that presented as an asymptomatic solitary tumor witha rapid growth on the nose of a 16-year-old boy. Routine microscopic histopathological evaluation of the patient’s skin biopsy showed numerous eosinophils and Touton giant cells. Immunohistochemical evaluation was positive for CD68 and factor XIIIa. We performed extended excision to prevent recurrence and the tumor did not recur after resection.