Much confusion surrounds a rare and occasionally described variant of Kaposi’s sarcoma (KS) known as lymphangioma-like or bullous KS. We describe the typical clinical and histologic features and elucidate the etiologic cell in lymphangioma-like KS. A computer-based review of the English-language literature was performed. Routine histologic and immunoperoxidase techniques were performed on formalin-fixed tissue. Immunoperoxidase staining for anti-CD31, anti-CD34, and anti-factor VIII–related antigen suggests that the etiologic cell of origin is the vascular endothelial cell. Lymphangioma-like KS has been described for more than a century and given various names. Dilated vascular spaces correlate with the clinically bullous lesions, which are veritable KS and not a secondary reaction to it. (J Am Acad Dermatol 2000;43: 123-7.)