Background: AIDSrelated Kaposi’s sarcoma (KS) is an AIDS-defining illness and is now increasingly recognized in children infected with HIV. Many of these cases are missed due to low index of suspicion. Vertical transmission of HIV is the commonest route of transmission in children and this is preventable by early maternal antenatal diagnosis, early commencement of HAART by the mother and adoption of the safest possible mode of delivery. The index case did not benefit from these services, making her acquire HIV and then develop AIDS-defining illness (KS) at a tender age of 4 years.Aim and objective: The study is aimed at emphasizing the need for all pregnant women to have antenatal screening as soon as pregnancy is confirmed so as to prevent vertical transmission of HIV to the unborn child. It is also aimed at creating awareness in clinical practice so as to increase index of suspicion among clinicians when evaluating chronically ill children.Case Description: GG was a 4year old girl who presented at 108 Nigerian Air force Hospital, Abuja with non-itchy skin rashes of two weeks duration, generalized body swelling, cough and haematochezia of one week duration, and fever of four days duration. She was a paternal orphan having lost her father a year prior to presentation to Acquired immunodeficiency syndrome (AIDS)-related illness. Her mother also tested positive to Human Immunodeficiency virus (HIV) and has been on highly active anti-retroviral therapy (HAART) for a year. Physical examination revealed moderate pallor, bilateral pitting pedal oedema, and matted non-tender peripheral lymphadenopathy. There were papular and nodular skin lesions with a reddish solid lesion on the hard palate. She tested positive to HIV. Abdominal ultrasound scan revealed moderate hepatomegaly with ascites, while chest x-ray showed bilateral interstitial pneumonitis, right hilar lymphadenopathy, right pulmonary nodule and ipsilateral pleural effusion. Excisional biopsy of one of the skin nodules confirmed the diagnosis of Kaposi’s sarcoma. She was commenced on HAART and antibiotics, with a unit of blood transfused. Her condition deteriorated, which necessitated referral to university of Abuja Teaching Hospital where she eventually died after a day of admission.Conclusion: Paediatric AIDSrelated KS is becoming increasingly common in Nigeria and as such, high index of suspicion is required while evaluating pediatric patients. This, coupled with early commencement of HAART is required to avert the pitfalls observed in this case; bearing in mind that childhood AIDSrelated KS is lethal.Keywords: Generalized lymphadenopathy; Kaposi’s sarcoma; Missed opportunity; Paediatric AIDS; Skin rashes