Pituitary apoplexy, a rare but life-threatening condition, may be highly variable in its clinical appearance and therefore should be considered in any patient with abrupt neurologic deterioration. We reviewed the literature on acute massive pituitary infarction to create an awareness of predisposing factors, the pathophysiologic mechanisms responsible for its heterogeneous manifestations, and possible options for investigation and management. These concepts are reinforced by examining the course and outcome of a rare case of pituitary apoplexy manifesting a full range of neurologic and endocrine abnormalities.