Situs inversus totalis (SIT) is a rare condition in which there is complete right to left reversal of the abdominal and thoracic organs. SIT generally does not bear any pathophysiological significance, and the survival rate of patients with SIT does not differ from that of healthy individuals. However, patients with SIT require a thorough radiological examination to identify the presence of associated anatomic variations before undergoing invasive procedures such as surgery or hemostasis of gastrointestinal hemorrhage because they may have accompanying abnormalities in anatomical structures along with reversed organs. Percutaneous endoscopic gastrostomy (PEG) is a relatively safe procedure that is most commonly performed for the enteral feeding of patients with dysphagia and a normal gastrointestinal function. However, the procedure requires extracaution because minor complications may lead to life-threatening situations due to the underlying illnesses. Here, we report the case of a patient with SIT who underwent a PEG procedure without complications, and review the existing literature on this subject.