A 9-year-old girl presented with failure to thrive, chronic mucopurulent nasal discharge, recurrent skin pustules and recurrent episodes of purulent ear discharge since 2 years of age. She had coarse facial features with extensive eczema, multiple pyoderma scars, florid dental caries, retained primary dentition, hypermobile joints and a woody induration of the vulva. Autosomal dominant hyper-IgE syndrome was suspected and confirmed by very high serum IgE levels. Vulval biopsy revealed a premalignant condition. STAT 3 mutation, which is usually responsible for this condition, was not found in our case, indicating an as yet unidentified mutation. The child also had unusual features like the total absence of clinical and radiological features of pneumonia. The premalignant change in the vulva was also unusual since vulval carcinoma has not been reported so far in children with this disorder. This child will require a close follow-up to look for malignant transformation.