Neurofibromatosis Type 1 (NF1) is one of the most common autosomal dominant diseases affecting multiple systems including the vascular, skeletal, and central nervous system. Noonan syndrome (NS) is an autosomal dominant genetic disorder, associated with musculoskeletal and skin manifestations. Coexistence of central giant cell lesions in patients with both NS and NF1 were reported in the literature. Development of multiple central giant cell lesions in a patient with a Noonan syndrome has been referred to as Noonan-like syndrome. A few cases with features of NF1 and NS have been termed as NF1-NS. Here, we present a case of so-called NF-NS associated with a central giant cell lesion.