A 7-month-old male patient presented with a light brown colored bean-sized macule on the lower back from birth. It had multiple satellite lesions. Her medical history was non-specific. Histological findings showed foamy histiocytic collection in dermis with many multinucleated giant cells. Based on the clinical and histopathologic manifestation, she was diagnosed as having a juvenile xanthogranuloma. Juvenile xanthogranuloma, a variant of non-Langerhans’ cell histiocytosis, is characterized by yellow, red or brown colored, firm to rubbery, papules or nodules in infants. Typical manifestations would help make an easy diagnosis. But atypical cases were also reported, mimicking dermatofibroma, keloid, pyogenic granuloma, keratoacanthoma, and so on. In this case, it was presented as a nevus lipomatosus superficialis or connective tissue nevi.