SummaryBackground:Spontaneous pneumomediastinum is an uncommon benign condition that is occasionally associated with subcutaneous emphysema and occasionally with pneumothorax, but is rarely associated with pneumorrhachis (air within the spinal epidural space).Case Report:We describe the case of a 20-year-old man and discuss a classification system of pneumorrhachis and its pathoanatomy, clinical and radiological presentation and management based on a detailed review of the previous literature. The pathophysiology is multifocal and diagnosis is state-of–the-art, as free intra-spinal air collection and coexistence of it both should be differentiated. Computed tomography with reconstruction of imaging is the method of choice for investigation. Symptoms associated with pneumorrhachis are due to its cause and origin and rarely due to pneumorrhachis, itself. Neurological symptoms and signs due to pressure effect are rarely found, but were present in our case. The management requires a multidisciplinary regimen and has to be individualized. The case was successfully managed conservatively, except for intercostal drainage for symptomatic pneumothorax. The patient stayed at rest and his symptoms improved within a few days. Seven days later the intraspinal air and pneumomediastinum were spontaneously resolved on follow-up chest computed tomography. In spontaneous pneumomediastinum, pneumorrhachis is self-limiting and benign.Conclusions:The same management is advised in spontaneous pneumomediastinum with or without pneumorrhachis in non-complicated, asymptomatic cases.