A 32-year-old woman presented at our clinic with a moderately soft, nontender, subcutaneous mass in the left temporal region and white hairs overlying the swelling. The lesion was surgically excised, and there was no recurrence at one year postoperatively. On histological examination, the hair follicles overlying the subcutaneous mass were devoid of pigment and a poorly demarcated fibrocellular process was seen in the subcutaneous mass, which was typical of neurofibroma. To our knowledge, only one case of poliosis associated with neurofibroma has been previously reported.