Abstract: Mediastinal Hemolymphangioma, as a rare clinical case, often presents atypical clinical manifestations and imaging features, which can easily be confused with other mediastinal lesions and lead to a high rate of misdiagnosis. This study aimed to deepen the clinical understanding of this disease by analyzing the case data of a patient with mediastinal hemolymphangioma that was confirmed by pathology in our hospital. Imaging examinations showed that the lesion localized to the anterior and middle mediastinum and presented as a space-occupying lesion with spot-like calcification foci visible inside. The lesion was adjacent to the superior vena cava, which showed dilatation, and extended and grew along the cardiovascular course towards the right atrium.