Anastomosing haemangioma is a rare subtype of capillary haemangioma. Pathologically, anastomosing haemangioma presents with anastomosing sinusoidal capillary-sized vessels in an architecture reminiscent of the splenic parenchyma. Its anastomosing architecture pathologically can lead to concern for angiosarcoma. Many cases of anastomosing haemangioma, which often occurred in the retroperitoneum, were well circumscribed, hyperdense on plain CT, revealed avid contrast enhancement and some of them exhibited fatty changes. In cases of tumours with fat of retroperitoneal occurrence, images frequently do not allow for easy differentiation from liposarcoma. Although anastomosing haemangioma with fatty changes and liposarcoma can be difficult to differentiate, no previous report has addressed this diagnostic difficulty. We have encountered a case of anastomosing haemangioma with fatty changes occurring in the perirenal space that was difficult to differentiate from liposarcoma. With retroperitoneal tumours accompanied by fatty changes and including a strongly enhanced area, the possibility of anastomosing haemangioma-which is a benign tumour-may also be considered. In such cases, biopsy is an effective means of diagnosis.