Background: Invasive pneumococcal disease (IPD) is a serious preventable disease-causing significant mortality and morbidity among children below five years of age.Methods: A five-year retrospective study involving hospitalised children aged below 14-years-old with IPD to two tertiary centres of two different states in Malaysia were conducted between year 2012- 2016. IPD was defined as isolates of Streptococcus pneumoniae from normally sterile body sites. This article reviewed previous hospital-based studies on paediatric IPD in Malaysia to provide an overview of the disease over the last three decades. A comparison was also made with the current study. Results: A total of 54 children were identified with IPD in this study. Sixty per cent of the study population was below the age of two-years-old. A significantly higher number of IPD cases were found among the indigenous group. Both spectrums of malnourished children were also susceptible to the infection. This study found that the majority of children developed septicaemia (43/54, 80%), followed by pneumonia (34/54, 63%) and meningitis (13/54, 24%). Pneumonia (p=0.001) and meningitis p=0.03) were more likely to occur in children less than one-year old. The study also showed that meningitis (p=0.01) was significantly associated with fatal cases. The second component of this article focussed on the review of the disease in Malaysia. There were 8 articles included, of which four each were clinical and microbiological studies. The review concurred that the young ones were vulnerable to the disease. The overall antibiotic resistance in Malaysia was low but on an increasing trend (10-30%). The mortality documented were only from the clinical studies, which showed a disparity of fatality rates in the different regions studied. The rate was low in Selangor (0-11%), in contradiction of a higher rate in Perak, the less developed area (26%). Conclusions: The study highlighted that the younger age group, extremes of nutritional status and indigenous children were at risk of IPD. On the literature review of paediatric IPD in Malaysia, there was inadequate information on the disease. A concerted effort to better describe the disease is needed.