Disruption of the myocardial architecture in left ventricular noncompaction (LVNC) may alter myocardial deformation. We evaluated LV myocardial deformation and tested the hypothesis that tight systolic-diastolic coupling occurs in LVNC. Longitudinal and circumferential strain and strain rates (SRs) as determined by speckle tracking echocardiography in nine children aged 5.6 ± 5.5 years was compared with those in nine controls. Left ventricular systolic myocardial deformation parameters were correlated with ejection fraction and indices of diastolic deformation. Compared with controls, patients had lower global LV systolic longitudinal strain (P = 0.008), systolic SR (P = 0.05) and early diastolic SR (P < 0.001). Similarly, LV systolic circumferential strain (base, P = 0.04; papillary muscle level, P = 0.01; apex, P = 0.04), systolic SR (base, P = 0.04) and early diastolic SR (papillary muscle level, P = 0.004, apex, P = 0.02) were lower in patients than in controls. Among patients, the LV ejection fraction correlated with global longitudinal systolic strain and SR and circumferential systolic strain and SR at all levels (all P < 0.05). Positive correlations existed between early diastolic and systolic SRs in corresponding dimensions (longitudinal r = 0.80, P = 0.01; circumferential at base, r = 0.91, P = 0.001; papillary muscle level, r = 0.96, P < 0.001; apex r = 0.98, P = <0.001). In conclusion, LV myocardial deformation is reduced in the longitudinal and circumferential dimensions and manifests tight systolic-diastolic coupling in children with LVNC.