The clinical and pathological findings in 4 cases of primary giant cell carcinoma of the larynx are reported. This variant of large cell carcinoma resembles the well-defined giant cell carcinoma of the lung. It has to be distinguished from other types of carcinoma of the larynx. This can be difficult on small biopsy specimens and the definite diagnosis may only be established after the detailed examination of the excised tumour. The highly malignant behavior of the laryngeal type was noted. To our knowledge this is only the second report on giant cell carcinoma of the larynx.