The authors present a rare clinical case of a patient with Bardet-Biedl syndrome and chronic kidney disease, who reached end-stage renal disease (ESRD) and underwent a long-term hemodialysis treatment, during which infections with Hepatitis C Virus (HCV) infection and Cytomegalovirus (CMV) infection were established. Kidney transplantation from an alive unrelated donor was performed. Later, an adenocarcinoma of the esophagus was diagnosed at an early stage, treated surgically with resection of the esophagus and gastroesophagoplasty afterward. Seven months later, a rare complication of the immunosuppressive therapy with Cyclosporin A occurred, which consisted of spontaneous bilateral pleural hemorrhage. The same, as well as the postoperative ventral hernia, was successfully resolved. Concomitant HCV was also treated. Rare autosomal recessive syndrome with severe complications, adenocarcinoma of the esophagus, spontaneous bilateral pleural hemorrhage after the operation, and successful treatment were discussed.