Introduction/Background Vulvar basal cell carcinoma is rare vulvar neoplasm accounting for less than 5% of all vulvar neoplasms. Vulvar basal cell carcinoma is usualy an indolent and destructive tumor that rarely metastases.

Methodology We retrospectively analyse clinical features and management of patients diagnosed with rare vulvar tumor – basal cell carcinoma (BCC) at the University Medical Centre Ljubljana in the last 5 years. A total of 10 patients were identified.

Results Mean age at diagnosis was 75,6 years. All of the patients were caucasians. All of the patients presented with vulvar lump. Majority of tumors were histologically defined as nodular type or superficial type. All patients were managed surgically with wide local excision. We observed two recurrences in our case series. In one recurrence was local and the patient was treated with wide local re-excision. In the second patient recurrence was observed with unusual location in para aortic lymph nodes.

Conclusion According to literature review this is the first case of recurrence of vulvar basal cell carcinoma in paraaortic lymph nodes. Basal cell carcinoma tends to grow locally in a destructive pattern and metastatic spread is rare.