Acute distal biceps tendon (DBT) pathology includes bicipitoradial bursitis, tendinosis, partial and complete tears. Diagnosis of complete DBT tears is mainly clinical, whereas in partial tears medical imaging is a valuable addition to the clinical diagnosis. New insights in clinical and medical imaging of partial tears may reduce time to diagnosis and may guide the treatment plan. Most complete tears are best treated with primary repair using either a single-incision or double-incision approach with good clinical outcome. The double-incision technique has a higher risk of heterotopic ossification, whereas a single-incision technique carries a higher risk of nerve-related complications. Intramedullary fixation may be a viable solution to negate the risk of posterior interosseus nerve lesions in single-incision repairs. DBT endoscopy can be used to treat low-grade partial tears and tendinosis. Cite this article: EFORT Open Rev 2021;6:956-965. DOI: 10.1302/2058-5241.6.200145