We report the second known case of yolk sac tumour of the external auditory canal, occurring in an eight-month-old girl. The excised tumour demonstrated histopathological and immunohistochemical features identical to those of yolk sac tumours of gonadal origin. The tumour was aneuploid by flow cytometry. The patient received chemotherapy post-operatively and has had no evidence of disease 13 months after surgery.