Multiple adult xanthogranuloma (MAXG) is an uncommon adult form of xanthogranuloma. The clinical manifestation of MAXG is relatively rare, but it has characteristic histopathological and immunohistochemical features. MAXG is mainly associated with cutaneous damage, and extracutaneous manifestations such as cervical spine, intracardiac, and periocular lesions have rarely been reported. In patients with systemic association, clinicians should pay attention to the possibility of hematological malignancies. We report the case of a 17-year-old man with multiple lesions on the head, face, neck, arms and trunk, along with the clinical manifestations and pathological features.