Abstract:An 8‐year‐old girl had small, papular vulval lesions for six years; the lesions were yellowish with numerous surface depressions. Symptoms due to the action of mastocyte mediators were observed. A biopsy specimen showed a dense monomorphous infiltrate of the deep dermis by rounded cells with granular cytoplasm and a round or oval central nucleus. The morphology of the lesions and red‐purple metachromatic staining led to the diagnosis of xanthelasmoid mastocytosis. Symptoms were controlled with hydroxyzine. Annual follow‐up has shown no evidence of systemic involvement to date. Surgery should be contemplated as a future therapeutic option, in view of the location of the
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