We report the ninth case of plexiform neurofibroma of the larynx, which occurred in a 2-year-old with multiple café au lait spots and obstructive sleep apnea. In discussing this clinical problem, we have attempted to make the following points. 1) A patient's having more than six café au lait spots of greater than 1.5-cm diameter is diagnostic of von Recklinghausen's disease. 2) It is much more difficult to completely excise plexiform neurofibroma than nonplexiform neurofibroma. 3) The association of juvenile xanthogranuloma with von Recklinghausen's disease may be a risk factor for the later development of leukemia.
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