The Headache and Neurologic Deficits with cerebrospinal fluid Lymphocytosis syndrome (HaNDL) is a rare, benign and self-limited entity of unknown cause, diagnosed by exclusion. It usually presents with neurological deficits such as hemiparesthesia, dysphasia and hemiparesis. However, seizures are not usually associated with the clinical spectrum of this syndrome. Here we report a case of a 35 year-old male patient with multiple episodes of moderate-severe headaches with transient hemiparesthesia, dysarthria, confusion, visual hallucinations, disinhibited behavior, and a bilateral tonic-clonic seizure. HaNDL diagnosis was made after clinical improvement and CSF evolution. Clinicians should consider HaNDL syndrome in patients presenting with headache, seizures, and confusion, when all other etiologies are ruled out.