To elucidate the relationship between regional myocardial adrenergic innervation and left venticular hypertrophy in patients with hypertrophic cardiomyopathy (HCM), simultaneous iodine-123 metaiodobenzylguanidine (I-123-MIBG) and thallium-201 (Tl-201) myocardial scintigraphies were performed in 21 patients with HCM. In 7 patients both I-123-Tl-201 images showed a normal distribution pattern, and in 13 patients Tl-202 showed a normal distribution, but I-123-MIBG showed a low distribution or defect. Myocardial uptake of I-123-MIBG was reduced even in the segments where myocardial hypertrophy was confirmed by the echocardiographic method. The incidence of a low distribution or defect in I-123-MIBG was significantly higher in the posterior, lateral and apical segments than in the anterior and septal segments in HCM patients (each, p <0.01). The difference in the distribution pattern between I-123-MIBG and Tl-201 may reflect adrenergic nerve dysfunction. The disorder of adrenergic innervation seems to precede myocardial damage. From these findings, we conclude that a good correlation between myocardial adrenergic innervation and left ventricular hypertrophy is not necessarily observed, and the difference in distribution pattern between I-123-MIBG and Tl-201 usually takes place in the advanced stage of HCM.