Giant hepatic angiomylipoma presenting with painful hepatomegaly.

Hepatic angiomyolipoma (HAML) is a rare mesenchymal neoplasm belonging to the perivascular epithelioid cell tumor (PEComa) family. Although usually asymptomatic and incidentally detected, large lesions may cause symptoms and pose diagnostic challenges. We report the case of a 69-year-old man presenting with painful hepatomegaly. Computed tomography revealed a 12-cm, predominantly fat-containing hepatic mass. Percutaneous biopsy demonstrated characteristic morphological features and an immunohistochemical profile showing HMB-45 and Melan-A positivity, with negative S100 and smooth muscle actin staining in the epithelioid tumor cells. Surgical resection was performed, with a favorable postoperative course. This case highlights the importance of integrating radiological and histopathological findings for accurate diagnosis and individualized management of giant hepatic angiomyolipoma.