Calcium pyrophosphate crystal deposition (CPPD) associates with ageing, osteoarthritis (OA), uncommon metabolic diseases, mutations and polymorphisms in the ankylosis human gene (ANKH). CPPD is frequently polyarticular, occurs due to a generalised articular predisposition, and the association between CPPD and OA is joint specific, for example CPPD associates with knee OA, but not with hip OA. Other recently identified associations include knee malalignment (knee CC), low cortical BMD and soft-tissue calcification. CPPD is generally asymptomatic. A recent study reported that knees with OA plus CC at the index joint, or at distant joints (in absence of index joint CC), were more likely to have attrition. CPPD can cause acute CPP crystal arthritis, chronic CPP crystal inflammatory arthritis, and is frequently present in joints with OA. Joint aspiration remains the gold standard for diagnosing CPPD, although other promising techniques are emerging. Patients with polyarticular or young onset CPPD should be screened for underlying metabolic abnormalities, however, such testing can be unrewarding. The treatment of CPPD is symptomatic. Acute CPP crystal arthritis is treated with rest, local application of ice-packs, joint aspiration, colchicine and/or intra-articular corticosteroid injection (once infection is excluded). Colchicine, low-dose corticosteroids, hydroxychloroquine and radiosynovectomy are recommended for the treatment of chronic or recurrent acute CPP crystal arthritis. Recent RCTs did not confirm any benefit from methotrexate, and although there is increasing interest in the use of anti-IL1 agents for acute or chronic CPP crystal arthritis, their efficacy has not been formally examined. Unlike gout, currently there are no treatments to eliminate CPP crystal deposits.