Aim of this review is to discuss recent findings concerning the management of stroke-like episodes (SLEs) in patients with mitochondrial disorders (MIDs). Various databases were searched for appropriate literature. SLEs are a dominant feature of MIDs and occur most frequently in MELAS-syndrome, less frequently in MERRF-syndrome, Kearns-Sayre-syndrome, or Leigh-syndrome. SLEs occur at all ages and are frequently accompanied by other cerebral abnormalities. Clinically, SLEs mimic ischemic stroke but not on imaging studies and concerning the management. The morphological equivalent on MRI is the stroke-like-lesion, representing a vasogenic edema (hyperintensity on T2, diffusion-weighted imaging and apparent diffusion coefficient in the acute and subacute or chronic stage, most frequently in the parieto-occipital region, surpassing vascular territories). For diagnostic and therapeutic reasons SLEs need to be clearly delineated from ischemic stroke and cerebral bleeding. Though there is no causal therapy available, symptomatic and general measures can help to resolve the clinical manifestations. In conclusion this review shows that SLEs are a dominant feature of some syndromic or non-syndromic MIDs. The most effective strategy for the treatment of SLEs appears to be the application of L-arginine, coenzyme-Q, steroids, edaravone, creatine-monohydrate, or dichloracetate.