Diabetic ketoacidosis has been described in the literature as a rare possible initial presentation of acromegaly before a diagnosis of acromegaly is eventually made. Indeed, diabetic ketoacidosis is a recognised complication of acromegaly. There are a number of factors that can predispose patients with acromegaly to diabetes as well as to diabetic ketoacidosis. These include high levels of growth hormone and insulin-like growth factor 1 in acromegaly and the effect on glycaemia by medications used in the management of acromegaly. Ketoacidosis has been described in patients with acromegaly even without the presence of an underlying autoimmune diabetes. Patients with acromegaly and ketoacidosis often respond to treatment and may not require long-term insulin.