Primary hepatic myelolipoma is a rare hepatic mesenchymal tumor mixed by adipose tissue and bone marrow, and there is a lack of general guidelines related to its epidemiology and clinical management.

A 26-year-old woman was admitted to our department complaining of a newly found tumor on her left lobe of the liver. The tumor was painless and not associated with any systemic or localized compressive symptoms. Serological tests disclosed a slight increase of gamma-glutamyl transpeptidase (70.0 U/L) and total bilirubin (19.2 μmol/L). Ultrasonography showed a mass about 5.0 cm × 5.0 cm in size that was located in the left lobe of the liver and displayed hyperechoic and well-encapsulated characteristics. Surgical resection was applied, and the following histopathological examination observed a variable proportion of myeloid tissues scattering throughout mature fibrotic adipose tissues, in which myeloid, erythroid, and megakaryocytic cells can be found in magnified view. The follow-up did not show any changes 6 mo after surgery.

This case highlights an extremely rare hepatic mesenchymal tumor, the primary hepatic myelolipoma, and discloses the common characteristics behind this disease and gives clinical recommendations.