Background: Recently, several cases of atypical left ventricular (LV) ballooning syndrome without involvement of apex have been reported. However, assessment of the clinical features of this novel presentation and comparison with typical transient LV apical ballooning syndrome has not been performed. Methods and Results: Over a 5-year period, 14 patients were diagnosed with LV non-apical ballooning syndrome in our hospital. The diagnostic criteria were (1) LV ballooning without involvement of the apical segment, (2) absence of significant coronary artery disease as assessed by coronary angiogram, and (3) electrocardiographic changes. As a control group, 29 patients who were diagnosed with classical LV apical ballooning syndrome during the same period were enrolled. Baseline characteristics were similar between non-apical ballooning (group NA) and apical ballooning (group A) patients except there was tendency for the NA group to be younger (median age 60 versus 70, P=0.056), and fewer patients had hypertension in group NA than in group A (14% versus 62%, P=0.003). Clinical presentations and in-hospital course of illness were also similar between the groups, but electrocardiograms showed a higher frequency of T-wave inversion in group A than in group NA (97% versus 64%, P=0.01). Most patients improved rapidly with restoration of LV function. Conclusions: We described the clinical characteristics of LV non-apical ballooning syndrome. The clinical presentation of this novel syndrome was mostly similar to classical LV apical ballooning syndrome except for electrocardiographic changes, which indicates that the 2 syndrome may be a single syndrome with different manifestations.