Ectopic liver (EL) is a rare congenital condition in which liver tissue develops outside the main liver without any anatomical connection to it. While typically an incidental finding during imaging or surgery, EL can occasionally present with symptoms, especially when complications like torsion, bleeding, compression, obstruction, or even neoplastic changes (benign or malignant) occur. Diagnosis often starts with imaging techniques like ultrasound, CT, or MRI, but confirmation usually requires a biopsy. This case study reports on a 22-year-old Italian female who presented with acute abdominal pain. Initial abdominal ultrasound and contrast-enhanced ultrasound (CEUS) using Sonovue®, followed by a CT scan and an ultrasound-guided biopsy, suggested the presence of a hepatocellular adenoma (HCA) arising from ectopic liver tissue. Due to the symptomatic nature and diagnostic findings, laparoscopic excision was performed, and histological examination confirmed the diagnosis of hepatocellular adenoma in the ectopic liver. This case highlights the essential role of ultrasound and advanced imaging in detecting and characterizing EL, especially when associated with potentially symptomatic or neoplastic transformation. Ultrasound, including CEUS, provides a detailed initial assessment, aiding in the identification of EL’s unique vascular and structural features. However, imaging alone may not fully establish a diagnosis, and biopsy is crucial for confirming HCA or ruling out malignancy. This report underscores the importance of a multi-modality imaging approach in the diagnostic pathway for symptomatic EL, supporting surgical intervention in cases with acute symptoms or suspected neoplastic changes.