Atypical chronic myeloid leukemia (aCML) is a neoplasm with poor prognosis, characterized by myeloid hyperplasia, dysmyelopoiesis and absence of BCR-ABL1 gene. Clinically, the disease course may be similar to chronic myeloid leukemia (CML), BCR-ABL1 positive. It presents in seventh to eighth decade of life with few cases of paediatric aCML being reported. Here, we report a case of aCMLin a 5-month baby who presented with massive splenomegaly. The diagnosis was in accordance with the WHO criteria established in 2008.
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