We present a case of a young woman with Birt-Hogg-Dubé syndrome and multiple neurilemmomas involving different nerves in a single extremity. Birt-Hogg-Dubé is an uncommon but increasingly recognized syndrome. Hand surgeons may wish to consider this disease when faced with a patient with multiple neurilemmomas, especially when associated with renal tumors, spontaneous pneumothoraces, or other benign or malignant neural or non-neural tumors.
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