A 69-year-old male patient underwent excision of hidradenitis suppurativa (HS) affecting both gluteal areas and the perineum. The perineal specimen contained a 1-cm superficially invasive, well-differentiated keratinizing squamous cell carcinoma. The patient was free of recurrence 1 year after surgery. A 66-year-old male patient was diagnosed with massive perineal HS more than 40 years previously. More than 30 abscesses and suppurative sinus tracts were surgically treated over the years. He eventually died of unresectable pelvic squamous carcinoma. Search of the literature and available bibliography revealed 47 retrospective studies of skin carcinoma arising in HS since 1959, including a total of 64 patients together with the two patients treated by our team. Squamous cell carcinoma is a rare but potentially fatal complication of HS. Surgery is the only known treatment method that provides a real chance for cure for both HS and a carcinoma that complicates it. HS must be treated early with complete excision to avoid chronic progression of the disease that can cause cancerous degeneration. A high index of suspicion, early tissue diagnosis, and immediate referral for radical surgery carry the only hope for cure in those whose HS harbors malignancy.