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Basal cell carcinoma (BCC) of the vulva is rare, accounting for 4.9% of primary vulvar cancers in our material. We present a study of 45 of these cases. Patients' age at presentation ranged from 60 to 92 years (mean 76 years). Symptoms were not specific, the most common being vulvar pruritus, and no specific etiological factors were apparent. Delayed treatment was frequent. The gross appearance was of a nodule or polyp, ulcer, or area of pigmentation or depigmentation; few were diagnosed as BCC before microscopic examination. Histologically, almost half were of infiltrative type, none was of morpheic type, and six contained areas of adenoid BCC. There were lymph node metastases from one large, deeply invasive BCC. After follow-up of 1 to 112 months (mean 59 months), four (9%) tumors recurred, three of which had been incompletely excised. The metastasizing tumor recurred locally after 23 months, despite apparent complete excision, and resulted in the only death from disease. Unrelated disease killed 19 patients, and 25 were alive at last follow-up. Our findings indicate that vulvar BCC usually behaves in a benign fashion, but is potentially fatal.