The aim of this preliminary study was to provide descriptive neurocognitive and quality of life data for the purpose of exploring outcome trends among childhood patients with Langerhans cell histiocytois (LCH) involving the skull. Neuropsychology measures assessing IQ, academic achievement, memory, attention, psychomotor ability, objective behavioral measures and quality of life were administered to 11 LCH patients ages 3 to 15 at Childrens Hospital of Los Angeles diagnosed with either LCH involving the central nervous system (CNS), as indicated by the presence of diabetes insipidus, or skull lesion "isolated" forms of the disease. Performance results suggest that subsets performed similarly on neurocognitive measures, such as IQ and achievement, with a wide range of scoring within groups. Quality of life measure outcomes, although slightly lower than previous reports from healthy populations, were consistently high among the two subsets, suggesting positive treatment and post-treatment adaptation. Variation in study performance outcomes among patients draw attention to the need to individually identify and assess patients "at risk" for neurocognitive sequalae. Discussion centers on study design considerations for future research that is conducted with larger sample sizes.