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Neurofibromatosis type 1 (NF-1) predisposes individuals to the development of benign and malignant tumors. The association of NF-1, juvenile xanthogranuloma (JXG), and juvenile myelomonocytic leukemia has been described in the literature. It is unclear whether JXG alone constitute a risk factor for leukemia or other malignancies in children with NF-1.To determine if there is an association between NF-1, JXG, and malignancy.We conducted a retrospective case-control study comparing children with NF-1 and malignancy (cases) with sex- and age-matched children with NF-1 without malignancy (controls).We identified 739 patients with NF-1 over a 20-year period, 14 of whom also had a diagnosis of malignancy. These cases include 9 (64%) boys and 5 (36%) girls. JXG were found in 4/14 (28.5%) cases and 6/29 (21%) controls (odds ratio 1.5, 95% confidence interval 0.35-6.6, P = .56).Retrospective design, small number of cases, and inconsistent documentation of clinical findings, including age at disappearance of JXG.Juvenile xanthogranulomas do not appear to confer an increased risk for malignancy in children with NF-1.