A 20-month-old boy with anhidrotic ectodermal dysplasia developed fever and papulopustular skin lesions. A skin biopsy showed the histologic changes of acute miliary tuberculosis. Anhidrotic ectodermal dysplasia is characterized by an absence of seromucous glands in the oropharynx and tracheobronchial tree, making children with this disease prone to viral and bacterial respiratory infections. Mild defects in their immunologic system have also been reported. We suggest that these abnormalities may have predisposed the patient to this rare form of cutaneous tuberculosis.

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