Context: Atypical genital nevus is rare and the only poorly documented melanocytic lesion in the spectrum of nevi of special sites. It is characterized by unique and reproducible histologic features, which may appear alarming and may raise significant concern for a diagnosis of melanoma.
Objectives: To present the clinical and histologic features of atypical genital nevus with emphasis on distinction from vulvar melanoma.
Data sources: The findings are based on a review of the current literature.
Conclusions: Atypical genital nevus is most often found in the vulva of premenopausal women. Despite the alarming histologic features, its clinical behavior appears benign with only rare local recurrence after incomplete removal. Recognition of this unusual melanocytic lesion and its characteristic histologic features is important to avoid overdiagnosis of invasive melanoma, with subsequent wide excision or sentinel lymph node biopsy, especially in view of the sensitive anatomic location.