A 24-year-old right-hand dominant male with severe Hemophilia A presented with acute elbow pain, associated paresthesias, and weakness in the ulnar nerve distribution after upper body weight lifting. In the week prior, he missed three doses of Factor VIII replacement. After no improvement with conservative measures, he was taken to the operating room urgently for decompression and was noted to have a perineural hematoma in the cubital tunnel. At final follow-up, the patient reported complete resolution of symptoms. Acute cubital tunnel syndrome in hemophiliac patients that does not respond to medical treatment is best treated with surgical decompression using minimal dissection to prevent hematoma formation, preserve perineural membranous tissue, and avoid destabilizing the nerve.