Aim and objective: To present a case of acute respiratory obstruction due to isolated hemorrhage in the larynx in a patient with coagulation disorder, which is a very rare presentation. Background: Acute upper airway obstruction can occur due to various causes. Spontaneous, unprovoked hemorrhage in the larynx can be a significant and potentially life-threatening complication. Coagulation disorders constitute a small percentage of such cases. An accurate initial assessment of a struggling, dyspneic patient can make the diagnosis challenging. Case description: Here, we describe a case of spontaneous internal hemorrhage in the larynx leading to upper airway obstruction in a patient with hemophilia A. The patient was admitted to an intensive care unit for airway observation and treatment with factor VIII supplementation. In the first 24 hours, there was a rapid respiratory improvement. The laryngeal hematoma gradually resolved by the 5th day. Conclusion: The patient was managed conservatively, and no intubation or tracheostomy was needed. Clinical significance: Hemophilia A can lead to life-threatening airway obstruction due to isolated laryngeal hemorrhage. Examination and diagnosis can be challenging if the prior history is not known as the condition mimics various other conditions. We need to keep an eye on subtle pointers that instigate an episode of bleeding. Keeping a high index of suspicion with prompt airway control and rapid reversal of coagulopathy can be lifesaving.