Avascular necrosis of the capitate is rare. Little is known about the aetiology, disease progression or optimal management. From 1992 to 2012 we treated six patients; four had a scaphocapitolunate arthrodesis and two had a four corner arthrodesis. The average follow up was 9 years (range 1–20). Three patients had good or excellent results, two fair and one poor, based on a visual analogue scale for pain and satisfaction and a Quick-DASH score. The Mayo wrist score was satisfactory in five cases and poor in one. Better results were seen when the arthrodesis fused. In the English, French and German literature 42 other cases were found. The aetiology, patient characteristics, clinical presentation, treatment and outcome were reviewed. Type of study/level of evidence: therapeutic IV.