Snapshot A 3-year-old boy presents to his pediatrician for multiple hyperpigmented lesions on his skin. Family history is significant for a genetic disorder in which his father to have similar skin lesions that are much larger. Medical history is otherwise unremarkable. On physical exam, there are 7 café au lait macules about 6 mm in size throughout the body along with axillary freckling. Introduction Definition login to view 7 more bullets Etiology Pathophysiology login to view 2 more bullets Genetics login to view 2 more bullets Presentation Physical exam login to view 6 more bullets Studies Preventative login to view 1 more bullet Differential Neurofibromatosis type 2 login to view 7 more bullets DIAGNOSIS Making the diagnosis login to view 2 more bullets MRI Treatment Treatment is targeted to the clinical manifestations login to view 1 more bullet Complications Increased risk of malignancy Prognosis Associated with increased mortality