Celiac artery compression syndrome (CACS) and superior mesenteric artery syndrome (SMAS) are 2 rare diseases, widely described in literature. Their association has not been specifically investigated; in fact, few cases have been reported. For this reason we reviewed our experience from January 1974 to June 2004. We report 59 patients affected by CACS and 28 by SMAS. Coexistence of both syndromes in 8 patients was observed. These 8 patients were successfully treated with duodenojejunal bypass and decompression of the celiac trunk. In this paper, we analyze the pathogenesis, clinical presentation, diagnosis, and treatment of these syndromes, emphasizing their common aspects. The misdiagnosis of this association may justify in some cases the controversial results reported regarding the surgical treatment of these syndrome.