Massive plexiform neurofibromatosis is an uncommon manifestation of Type 1 neurofibromatosis. These tumours result in functional disability and severe disfigurement. Due to the variable natural history od these lesions, the optimal management is not well defined and surgery is often delayed until significant disfigurement has occurred. The aim of this report is to discuss the optimum timing of surgical intervention and to highlight the difficulties in dealing with an advanced lesion.