Two cases of sex cord tumour of the ovary with annular tubules were diagnosed in African females of 23 and 57 years. In one case it was associated with the Peutz-Jeghers syndrome and with Stein-Leventhal ovaries whilst in the other case, pigmentation of the oral mucosa was present but without demonstrable polyposis of the intestine. The patient with the Peutz-Jeghers syndrome presented with primary sterility whilst the other patient had a normal menstrual and obstetric history. Signs of virilization were not found in either case. The previously reported literature is reviewed and discussed.