Celiac axis compression syndrome (CACS) is a rare entity of mesenteric ischemia, secondary to inadequate blood supply to the intestine, resulting in weight loss because of postprandial abdominal pain. Superior mesenteric artery (SMA) syndrome is an uncommon cause of intestinal obstruction manifesting with epigastric pain, bilious vomiting, and postprandial discomfort. Although the coexistence of both syndromes is very rare and has been reported only in eight patients in the literature, the CACS as a rare etiology of SMA syndrome has not yet been reported. Herein, we describe an uncommon case of SMA syndrome secondary to the CACS. The 27-year-old woman presented with epigastric pain, postprandial vomiting, and rapid body weight loss. The diagnosis of SMA syndrome was made by hypotonic duodenography and multidetector computer tomographic angiography. The CACS was also suspected by multidetector computer tomographic angiography. Surgical intervention was performed and the presence of CACS was confirmed. Her symptoms subsided shortly after operation and she was in good health at 1-year follow-up.