We report the case of a 77 year old woman with a giant proliferating trichilemmal cyst of the scalp and a central squamous-cell carcinoma. Six months after radical tumor-excision and dissection of the locoregionary lymph nodes the patient developed retroclavicular lymph node metastases. The tumor develops from cells of the hair matrix and tends to recur after excision; in rare cases malignant transformation may occur. We suggest radical excision and complete histological examination of the tumor. In cases of malignancy, lymph node-dissection is sometimes necessary. In these cases close postoperative follow-up of the patient is mandatory.