The spleen plays a pivotal role in our immune system by facilitating the proliferation and differentiation of lymphocytes and monocytes. Typically located in the left upper quadrant retroperitoneally, splenic tissue found outside of its usual position is termed ectopic spleen. When the tissue maintains its histological architecture and encapsulation and receives blood supply from splanchnic vessels, it is called an accessory spleen. Although it commonly presents near the splenic hilum or pancreatic tail, rare instances have been reported in the gastric, liver, gastrosplenic/lienorenal ligaments, as well as thoracic and gonadal regions. However, the case of an accessory spleen, mimicking a hepatic lesion in the right diaphragm represents a novel observation.