Fracture of an ossification of the Achilles tendon (OAT) is a rare entity, and its etiology, pathology, and treatment remain unclear. We reviewed and scrutinized 18 cases (16 articles) of the fracture of an OAT. The most common etiologies of the ossifications include previous surgery and trauma. The fractures often occur without any trigger or with minimal trigger. The long, > 5 cm, ossification in the body of the Achilles tendon may have a higher risk of fracture. The OAT itself is often asymptomatic; however, its fracture causes severe local pain, swelling, and weakness of plantar flexion, which forces patients to undergo aggressive treatments. Regarding the treatments of the fractures, nonoperative treatment by immobilizing ankle joint could be an option for elderly patients. However, because it often cannot produce satisfactory results in younger patients, surgical treatment is typically recommended. Excision of the fractured mass and repairing the tendon is applicable if the remnant is enough. If there is a defect after the excision, reconstruction with autologous grafts or adjacent tendon transfer is performed. Gastrocnemius fascia turndown flap, hamstring tendon and tensor fascia lata are used as autologous grafts, whereas peroneus brevis and flexor hallucis longus tendons are used for the tendon transfer. If the fracture of an OAT is treated properly, the functional result will be satisfactory.