Pediatric stroke is a rare disease. Diagnosis and management are difficult because of the diversity of underlying risk factors, multiple differential diagnoses ("stroke mimics") and the absence of uniform therapy guidelines. Still, an early diagnosis is important to reduce mortality and morbidity by an appropriate treatment in specialized care centers. Clinical symptoms of neonatal stroke are often unspecific; main symptoms are epileptic seizures. In children hemiparesis, facial palsy and dysphasia are the most common presentations of stroke. Juvenile migraine is an important differential diagnosis and a stroke mimic. Hemorrhagic stroke in children is mainly caused by cerebral vascular malformations; the acute ischemic stroke (IS) in childhood is induced by non-arteriosclerotic arteriopathies, hematological, cardiac and metabolic diseases. In about 20% of patients the pathogenesis of the IS remains elusive. Gold standard of neuroimaging is the magnetic resonance imaging (MRI) and MRI angiography. In the acute situation the indication for the anticoagulation regimen is based on a preliminary assessment of the coagulation system. Subsequently, an extended laboratory diagnostics is needed for further evaluation of the stroke pathogenesis. For initial treatment unfractionated heparin in therapeutical dose, low molecular weight heparin (LMWH) or alternatively acetyl salicylic acid (ASA) can be administered. The indication for systemic or intraarterial thrombolytic therapy with recombinant tissue plasminogen activator (rt-PA) and for mechanical thrombectomy should be strictly verified in children. For these treatment options and secondary prophylaxis only a few therapy guidelines exist for children in contrast to adult stroke. In neonates with a first stroke without an underlying cardiac disease no anticoagulation or thrombocyte function inhibiting therapy is needed.