Acute megakaryoblastic leukaemia is an uncommon form of paediatric cancer. Extramedullary leukaemia in acute megakaryoblastic leukaemia is rare, which is reported to be associated with increased risk of induction death. This atypical presentation makes diagnosis difficult. Tissue biopsy from extramedullary site tumour can mimic other small blood cell tumours. This report describes a case of this rare presentation in a very young girl who presented with abdominal distension as if she had metastatic solid tumour.