Perivascular epithelioid cell tumor outgrowth from the liver

•Perivascular epithelioid cell tumor is a rare mesenchymal neoplasia and can be found in various body sites.•The diagnosis of hepatic PEComa is made by a positive immunohistochemical staining for HMB45 and Melan.•The diagnostic approach, treatment modalities, and follow-up procedures are not standard.•The main treatment modality for PEComa is surgical excision with adequate surgical margin. Perivascular epithelioid celltumor (PEComa) is a rare mesenchymal neoplasia and can be found in various body sites. On the other hand, hepatic PEComa is very rare, with only a few studies having reported hepatic malignant PEComa. There is no gold standard regarding the use of diagnostic imaging studies. The diagnosis of hepatic PEComa is made by a positive immunohistochemical staining for HMB45 and Melan A. Herein, we discussed the therapeutic and follow-up process of a symptomatic hepatic PEComa case. A 22-year-old woman presented with a palpable mass in abdomen. A computerized tomographic examination showed a giant hepatic mass of left lobe origin, which was excised surgically. The pathology result was reported as a PEComa. The diagnostic approach, treatment modalities, and follow-up procedures are not standard. The main treatment modality for PEComa is surgical excision with adequate surgical margin. A longer follow-up is required for patients with hepatic PEComa because the nature of the disease is not entirely clear.