Introduction: Lipomas are common benign mesenchymal tumors but are rarely encountered in the vulvar region. Their nonspecific presentation can mimic other vulvar masses, making accurate diagnosis important.

Objectives: To report a case of a lipoma of the labia majora and to systematically review the literature to characterize clinical presentation, diagnostic approaches, and management.

Methods: A systematic PubMed search was conducted from database inception through March 2026 using terms related to “lipoma” and “vulva.” Studies were included if they reported histologically confirmed vulvar or labial lipomas in human subjects. Non-vulvar lipomas, malignant tumors, and reports with insufficient clinical data were excluded.

Results: We report a case of a 55-year-old woman with a 9-year history of a painless, pedunculated right labia majora lesion that was successfully treated with excision and confirmed as a lipoma on histopathology. Our systematic review of vulvar lipomas identified 32 studies that demonstrated surgical excision as a consistently curative treatment, with no reported recurrences. Most reported cases occurred in adult women and involved the labia majora, with right-sided lesions more common than left. Tumor size varied widely. Imaging was frequently used but not universal.

Conclusions: Vulvar lipomas are rare benign tumors that typically present as slow-growing masses. Surgical excision with histopathologic confirmation is both diagnostic and curative, with excellent outcomes.