Kaposi's sarcoma (KS) of childhood is an extremely rare and unexplained disease. It is usually associated with immunosuppression and human herpes virus 8 (HHV-8) infection. It can involve skin, mucous membranes, lymph nodes and viscera. This investigation describes a case of a 3-year-old boy with cutaneous and lymphangiopathic KS. Initially, the patient presented with cutaneous lesions of KS behind the right ear. Two months later, the disease disseminated not only cutaneously but also to the cervical, axillary and inguinal lymph nodes. Immunological evaluation showed severe lymphocytopenia but without evidence of human immunodeficiency virus infection. The vascular nature of the lesion was supported by positive staining for CD31 and CD34. This study examines the clinicopathologic features of KS in children and summarizes the relevant literature.