BACKGROUND: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant vascular liver tumor diagnosed by histopathological evaluation. Standardized treatment is challenging because of its rarity; hepatectomy is preferred for solitary lesions and multiple transplantations. There is no consensus on the optimal treatment for HEHE; however, surgical excision is often considered effective. This report presents a case of initially suspected cholangiocarcinoma or renal cell carcinoma (RCC) metastasis, which was later confirmed as HEHE, with no recurrence during follow-up. CASE SUMMARY: A 52-year-old man with a history of left nephrectomy for RCC presented with an incidentally detected liver mass and nonspecific abdominal discomfort. Imaging revealed a 3-cm centripetal enhancing lesion in the right hepatic dome with indeterminate malignant potential. The patient underwent a laparoscopic right anterior sectionectomy and remained recurrence-free without complications during the 3-year follow-up period. CONCLUSION: Managing HEHE is challenging. Accurate diagnosis and surgical options, such as resection or transplantation, are essential with tailored multidisciplinary follow-up. The authors have read the CARE Checklist (2016) and the manuscript was prepared and revised according to the CARE Checklist (2016).