The association between Peutz-Jeghers syndrome and multiple epithelial neoplasias is infrequent. Tumors of the gastrointestinal tract, genitals and breast are common in this syndrome and tumors of the kidney and biliary tract are exceptional. We present the case of a 68-year-old woman with Peutz-Jeghers syndrome who presented with right kidney carcinoma and numerous small, bilateral tumors of the sex cords with annular tubules of the ovary. Right nephrectomy, double annexectomy and total hysterectomy were performed. The patient died two years later following the development of rectal carcinoma diagnosed by sigmoidoscopy and biopsy and an episode of obstructive jaundice compatible with cholangiocarcinoma.