As the most common form of skin cancer, basal cell carcinoma (BCC) is typified by locally infiltrative growth and a very low risk of metastasis. On occasion, however, this otherwise indolent neoplasm may behave aggressively, demonstrating deep tissue invasion and a high rate of postsurgical recurrence. The pathogenesis and determinants of such tenacious growth are not completely understood. Only 1% of all BCC's achieve the status of 'giant', as defined in 1988 by the American Joint Committee on Cancer. In this article, the authors provide a comprehensive review of the scientific literature on giant basal cell carcinoma (GBCC) of the skin and report their experience with this rare tumour subtype.