A rare case of kaposiform hemangioendothelioma in adult is reported. The 11x7x5 cm tumor was excised from deep subcutis of the abdominal region in 37-year-old man. No signs of Kasabach-Mer- ritt syndrome or lymphangiomatosis were present. Besides typical pattern of kaposiform heman- gioendothelioma, following unusual features were found: dilated vessels producing a gross impression of spindle cell hemangioma, areas of amianthoid-like fibrosis, and diffuse immunore- activity for CD99. The differential diagnosis included mainly spindle cell hemangioma (hemangi- oendothelioma), hemangiopericytoma-like solitary fibrous tumor, and Kaposi’s sarcoma.

        Key words: kaposiform hemangioendothelioma - spindle cell hemangioma - solitary fibrous tumor - CD99 - CD 34 - amianthoid-like fibrosis