The multiple naevoid basal cell carcinoma syndrome (Gorlin's syndrome) is an inherited multisystem disorder which affects not only the skin, but also the skeletal system and a variety of other tissues and organs. This is a report of the case of a 29 year old woman who presented with multiple advanced basal cell carcinomas. On the basis of a literature review, guidelines for management are suggested and the importance of early diagnosis and regular follow-up is emphasized.