In this case report, a 14-year-old male presented with episodes of migraine-like headaches preceded by unilateral hemiparaesthesia, hemiparesis, confusion, and dysphasia with the last two lasting more than four hours. The cerebrospinal fluid (CSF) showed lymphocytosis with no detectable aetiology, neuroimaging was normal, and an electroencephalogram showed diffuse slowing. The patient was diagnosed with headache associated with neurologic deficits and CSF lymphocytosis, i.e. syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis. The condition is a rare disorder, especially in children. Normally, a complete remission takes place within few weeks to months without treatment.