The etiologies of Keinböck's disease and intraosseous ganglion remain unknown. Both entities are rare and the coexistence of these two pathologies in the same patient and hand is even less frequent. We report the case of a 40-year-old man with a longstanding history of martial arts practice (karate) who developed an avascular necrosis of the lunate concomitant with a giant intraosseous ganglion of the scaphoid bone successfully managed by proximal row carpectomy. We review the literature of these two diseases.