A patient with gonadal dysgenesis was found to have a mucinous epithelial ovarian tumour on the left side and a streak gonad on the right. The preponderance of mucinous tumours (5 of 8) over other epithelial tumours in these patients is noted but the significance is not fully understood. Two models of pathogenesis (incessant ovulation and hypergonadotrophic hypogonadism) were proposed but neither satisfactorily explains the development of the tumours. Further ultrastructural, chromosomal and molecular biological study of these tumours may help to elucidate the underlying cause and pathogenesis.