An 18-year-old man with Gorlin's syndrome (the nevoid basal cell carcinoma syndrome) underwent excisional biopsy of a fibrous pseudotumor of the tunica vaginalis. Preoperative ultrasound confirmed the extraparenchymal nature of this paratesticular lesion, permitting a transscrotal approach. The pathophysiological considerations suggest that this finding (to our knowledge the first such case reported in association with Gorlin's syndrome) may be more than coincidental.