Introduction: Perianal tumours are a rare site of malignancy, and tumours primarily involving the perineal body without vaginal and anal canal involvement are uncommon.

Case summary: A 67-year-old woman presented with a lesion involving the perineum and rectovaginal septum without extension into vaginal or anorectal mucosa and with skip lesions in the vulva. Biopsy was confirmative of squamous cell carcinoma, with p16 positive. A complete metastatic workup with MRI of the pelvis and CECT thorax and abdomen was done. She was diagnosed with perianal carcinoma stage cT2N0M0 Stage II (American Joint Committee on Cancer 8th Edition of Cancer Staging) since the lesion reached the anal verge. Given the location of the tumour (perineal body), comorbidities and advanced age, she received radical radiotherapy with an intensity-modulated radiotherapy technique – 56 Gy in 28 fractions with the intention of organ preservation. The response assessment with MRI at 3 months showed a complete tumour response. She has been disease-free for 3 years and is on regular follow-ups.

Conclusion: Isolated perineal body squamous cell carcinomas are unusual, and synchronous vulvar skip lesion makes this case unique. Radical radiotherapy achieved organ preservation with tumour control and minimal toxicity in an elderly frail patient.