In Saudi Arabia, gastric cancer is a major health burden, ranking as the thirteenth most common cancer type. Situs inversus totalis (SIT), which is a very rare congenital anomaly, refers to a completely reversed location of the abdominal and thoracic organs (mirror image of the normal). Here, we present the first reported case of gastric cancer in an SIT patient in Saudi Arabia and the Gulf Cooperation Council (GCC) countries, and highlight the challenges faced by the surgical team in the removal of such cancer in this patient population.