Objective: We present the case of a 14-year-old Factor VIII-deficient patient with no history of trauma, who developed acute spontaneous compartment syndrome of the volar forearm. We also suggest a treatment strategy. Methods: Fasciotomy with hematoma evacuation and ipsilateral carpal tunnel release was performed, and the wound was closed with vascular loops in “Jacob's ladder” fashion. Factor infusions were continued overnight. Results: The volar forearm compartment was successfully decompressed, and the patient's coagulopathy was managed with appropriate clotting factors. Conclusions: Hemophilic patients warrant special consideration and multispecialty care; with replenished coagulation factors and timely surgical decompression, they can expect satisfactory recovery of muscular and neurological function.