Background: HIV associated KS is relatively rare in children and has been reported to be higher in East Africa compared to other regions. Literature on cases of histologically proven Kaposi sarcoma in children with HIV infection in West Africa is scanty. Case presentation: This communication presents three cases of KS seen among children in a paediatric HIV unit. The first case was an eleven year old HIV positive boy who had oral candidiasis that resolved with treatment and subsequently developed a painless, erythematous swelling at the middle of the dorsum of the tongue with central loss of papillae. He also had multiple discoid hyperpigmented flat lesions on the legs and soles of the feet. In addition to switching to second line antiretroviral therapy, he had chemotherapy. The lesions regressed. The second case was a double orphan who had KS involvement of the right eye, nasal cavity and lymph nodes. The tumour rapidly progressed and the child died before ART and chemotherapy could be commenced. The last case was a five year old girl with mild KS of the skin but also had other manifestations of severe HIV disease which she succumbed to. Incisional biopsies of the lesions revealed an invasive epithelial lined vascular tumour destroying the upper layers of skeletal fibres within the connective tissue stroma in keeping with KS. Human Herpes Virus type 8 (HHV8) screen was also positive for all the cases. Conclusion: A high index of suspicion must be entertained and biopsy of suspicious muco-cutaneous lesions is necessary to exclude a diagnosis of KS which is an indicator of severity and progression of HIV.