Fibromatosis is a rare disease with benign histology. Its infiltrative growth pattern may prevent complete resection. We report a case of a 40-year-old woman with fibromatosis of the neck requiring an emergency operation for acute upper airway obstruction. Gross residual tumour was left behind but excellent tumour shrinkage was achieved by using weekly low-dose methotrexate and vinblastine. Despite the use of newer agents such as imatinib, cytotoxic chemotherapy remains an efficacious treatment for inoperable fibromatosis.