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Pituitary gigantism is a rare condition associated with significant morbidity and early mortality if untreated. It should be considered as a differential diagnosis in tall patients with accelerated growth velocity. It is challenging to diagnose, as often the age of presentation overlaps with puberty, a time of natural increased growth velocity. This review aims to support general paediatricians in recognising and assessing these patients by summarising the aetiology, investigations, management and long-term outcomes of pituitary gigantism.