Although in literature almost 150 patients with Lance-Adams Syndrome (LAS) have been reported, neuroradiological evaluations were often performed in late stages and there is no serial study evaluating LAS from early stages. We herein report a serial neuroimaging study demonstrating early and transient involvement of cerebellum and thalami in a LAS patient. We may hypothesize that a transient cerebral hypoxia provoked a permanent synaptic rearrangements of the neuronal networks involved in the pathogenesis of post-hypoxic myoclonus in our patient.