BACKGROUND Bronchogenic cysts are rare congenital cystic lesions of foregut origin. They are the result of abnormal budding of the primitive tracheobronchial tube. Nonparasitic, true splenic cysts are another rare entity that might occur as incidental findings but may lead to deleterious courses in case of ruptures. METHODS We report here the first case of the uncommon association of a paraesophageal bronchogenic cyst and multiple primary splenic cysts in a 23-year-old woman suffering from dysphagia and chest pain. Successful complete resection of the paraesophageal cyst was performed using an abdominal, transhiatal approach and splenic cysts were subjected to organ-preserving TA stapler resection. CONCLUSIONS Thus far there is no proof or genetic indication for a direct association of bronchogenic cysts and multiple splenic cysts, however, the uncommon coincidence of both lesions in our patient might suggest a common origin. Difficulties in preoperative diagnosis, histopathological characteristics and surgical treatment modalities are presented.