Purpose To retrospectively summarize the clinical features of acromegaly complicated with fulminant pituitary apoplexy and analyze the prognostic factors to guide early identification and timely treatment of such patients. Methods A retrospective analysis was carried out to summarize the clinical manifestations, hormone changes, imaging, treatment and follow-up of 10 patients with acromegaly complicated with fulminant pituitary apoplexy admitted to our hospital from February 2013 to September 2021. Results The mean age of the 10 patients (5 males and 5 females) at the time of pituitary apoplexy was 37.1 ±13.4 years old. There were 9 cases with sudden severe headache and 5 cases with visual impairment. All patients had pituitary macroadenomas, of which 6 cases with Knosp grade ≥ 3. The level of GH/IGF-1 hormone after pituitary apoplexy was lower compared with pre-apoplexy, and 1 patient reached biochemical remission spontaneously. 7 patients underwent transsphenoidal pituitary surgery after apoplexy and 1 patient was treated with long-acting somatostatin analog. The biochemical remission rate was 37.5% in 8 patients immediately after treatment and 50% at the last follow-up. Patients with Knosp grade ≥ 3 were less likely to achieve biochemical remission than those with Knosp grade < 3 (16.7% vs. 100%, p=0.048), and patients who achieved biochemical remission had a smaller maximum tumor diameter [20.1（20.1,28.0）mm vs. 44.0（44.0,60）mm，p=0.016]. Conclusion Acromegaly complicated with fulminant pituitary apoplexy remains a diagnostic and therapeutic challenge.

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