A 46-year-old patient suffering from Hallopeau-Siemens type epidermolysis bullosa dystrophica presented a large squamous-cell carcinoma of the left hand. No metastases were observed. The tumour was resected and the phalanxes were amputated. The wound was covered with autologous skin grafts. Squamous-cell carcinoma is extremely aggressive in these patients and surgery is the only effective therapy. This life-threatening complication should always be kept in mind and a biopsy taken of every chronic, non-healing ulceration and of each atypical lesion.