Subhepatically located caecum and appendix is a very rare entity. It occurs due to the anomaly in fetal gut rotation that results in an incomplete rotation and fixation of the intestine. Appendicitis, which is a common surgical emergency, in combination with the abnormal subhepatic location, presents a great challenge in its diagnosis and management. Here, we describe a 42-year-old male with chronic dyspepsia who presented with sepsis and severe pain at his right hypochondriac and epigastric region. The final diagnosis was acute appendicitis of the subhepatic appendix. Our discussion focuses on the diagnostic approach and clinical and surgical management. We hope that our report will increase the awareness among the clinicians and hasten the management of such rare condition to avoid complications.