Hypothenar hammer syndrome is an uncommon cause of upper-extremity ischemia that is often overlooked in the absence of a thorough occupational and recreational history. Importantly, it is a reversible cause of hand ischemia that, if missed, can lead to significant morbidity and even amputation. The occupational ramifications and quality of life of those affected can be significant. Its relative rarity, set against the ubiquitous use of the hand as a 'hammer' is noteworthy. Several other causes of hand ischemia can present similarly; therefore, consideration of other etiologies must be thoroughly investigated. Key distinguishing clinical features, in addition to a detailed occupational and recreational history, may include characteristic sparing of the thumb, the absence of a hyperemic phase in 'Raynaud's phenomenon', and a positive Allen's sign. Both non-invasive and invasive diagnostic studies, including bilateral upper-limb segmental pulse volume recordings (PVR), arterial duplex examination, and upper-extremity angiography, are complementary to a thorough history and physical examination. Optimal management strategies are not well defined because of its rarity and resultant lack of quality, evidence-based data. Though most cases can be successfully managed non-operatively, micrographic arterial reconstruction may be limb saving in severe or recalcitrant cases. Newer, experimental strategies including selective sympathetic blockage using botulinum toxin A have been reported in a few recalcitrant cases. The brief case description illustrates the typical presentation and potential treatment strategies employed in a difficult case. A review of relevant literature is also presented.