Primary cough headache (PCH) is a rare headache disorder characterized by sudden, severe, bilateral pain precipitated by coughing or other Valsalva maneuvers, in the absence of intracranial pathology. It predominantly affects males aged 40–60 years, with a male-to-female ratio of approximately 3:1. The pathophysiology remains unclear but involves transient elevations in intracranial pressure, venous congestion, posterior fossa crowding, cerebrospinal fluid pressure gradients, and neural sensitization secondary to inflammation. Diagnosis necessitates detailed clinical history and neuroimaging to exclude secondary etiologies; MRI is instrumental in this differentiation. Indomethacin constitutes first-line therapy, effectively attenuating attack intensity and frequency. Alternative agents include acetazolamide and beta-blockers; therapeutic lumbar puncture may be considered. In refractory or contraindicated cases, non-invasive vagus nerve stimulation demonstrates preliminary efficacy. Accurate recognition of PCH enhances patient quality of life. Further investigation is warranted to clarify pathophysiology and refine therapeutic strategies.