Buschke-Löwenstein tumour (BLT) is rare and locally aggressive, and malignant transformation is a possibility. Because there is no consensus on the best treatment approach, the authors present a treatment algorithm based on several case reports. A 57-year-old male patient resorted to surgical consultation with a giant perianal cauliflower-like mass. A BLT was diagnosed. Due to the involvement of the anal sphincter, a wide local excision saving the rectum failed. Abdominoperineal resection was performed. Malignant transformation was diagnosed, and adjuvant radiotherapy was delivered. Clinical evolution was uneventful. Aggressive behaviour despite the absence of malignancy is the hallmark of BLT. The common presentation is an anal mass with a cauliflower-like appearance. Anal verrucous carcinoma and squamous cell carcinoma are the major differential diagnoses. BLT treatment is challenging. Surgery is the first-line treatment, raging from wide local excision to abdominoperineal resection. To improve outcomes, chemoradiation can be used in combination with surgery. Long-term follow-up is mandatory.