Infection by human herpes virus 8 (HHV-8) in childhood is common in the Mediterranean basin; however, classic Kaposi’s sarcoma (KS) is exceedingly rare in children not infected with HIV and not receiving immunosuppression, with only 30 cases reported since 1960. We recently reported two children with autosomal and X-linked recessive primary immunodeficiencies underlying KS in a context of multiple clinical manifestations. These reports suggested that classic KS in otherwise healthy children might also result from inborn errors of immunity more specific to HHV-8. In this paper, we describe three unrelated Turkish children with classic KS born to first-cousin parents. The first patient, a girl, developed KS at two years of age with disseminated cutaneous and mucosal lesions. The clinical course progressed rapidly and the patient died within three months, despite treatment with vincristine. The other two children developed a milder form of KS at the age of nine years, with multiple cutaneous lesions. A boy treated with interferon alpha therapy for 12 months is now in full remission at age 14, two years after treatment. The second girl is currently stabilized with etoposide, which was begun four months ago. None of the three children had any relevant familial history or other clinical features. The occurrence of classic KS in three unrelated Turkish children, each born to consanguineous parents, strongly suggests that autosomal recessive predisposition may drive the rare occurrence of HHV-8-associated classic KS in children.