“…The most common extracu taneous site is the eye (eyelid, limbus, iris, and optic nerve), but lesions have been reported within the heart, muscula ture, periosteum, pericardium, omentum, mucous mem branes, salivary glands, lung, liver, spleen and testes [4]. It is also associated with neurofibromatosis type 1 [50], juve nile chronic myelogenous leukemia [51], urticaria pigmen tosa [52], and cytomegalovirus infection [53], while sys temic JXG is known to occur without obvious cutaneous disease [39], Prior to this report, only 6 cases of symptom atic CNS involvement as part of systemic JXG have been reported to date [39], Symptoms have included diplopia, ataxia, diabetes insipidus, seizures, and recurrent subdural effusions. In these reports, radiographic abnormalities of the CNS were documented in the hypothalamus and third ventricle, lateral ventricles, cerebral hemispheres, brain stem, and cerebellum; although, only the report of Okubo et al [5] contains histologic confirmation of a true XG.…”