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Acromegaly is always complicated with comorbidities and increased mortality. The disease activity and mortality outcomes are highly correlated to the level of growth hormone and insulin-like growth factor 1. A variety of clinical manifestations of acromegaly have been reported. We present a unique case where a 49-year-old male was diagnosed with acromegaly with a first manifestation as an episode of diabetic ketoacidosis. Because he refused any suggestion of treatment, a recurrent episode of diabetic ketoacidosis with pituitary apoplexy occurred. A huge B-cell lymphoma displaying as a huge facial mass followed within 1 year of the diagnosis of acromegaly. Death from advanced cancer ensued 3 years later. This clinical experience strongly reinforces the urgency of controlling growth hormone and insulin-like growth factor 1 as soon as possible once acromegaly is diagnosed.