Ovarian tumours (or tumour‐like conditions) and Peutz‐Jeghers syndrome are associated more frequently than by mere chance. This seems to be particularly true for the “Sex cord tumour with annular tubules”, a new entity described by Scully in 1970. A case of this ovarian tumour found bilaterally together with a unilateral mucinous cystadenoma in a patient with Peutz‐Jeghers syndrome is presented with a discussion of the implications of this association.