Elbow chondromatosis is a rare benign disease that leads to the formation of intra-articular loose bodies, resulting in deterioration of joint functionality. Chondromatosis is divided into primary and secondary. Three phases of the pathology are recognized: depending on the stage, loose bodies may ossify and be evident on radiographic evaluation. Magnetic resonance imaging may aid in diagnosing cases without obvious radiographic evidence. Optimal treatment includes loose bodies’ excision and synovectomy. Both open and arthroscopic management seem to yield excellent outcomes, nevertheless, arthroscopic techniques are considered less traumatic, resulting in early rehabilitation and fewer wound healing problems. Thus, arthroscopic management of elbow chondromatosis has gained more popularity over the last two decades. Secondary elbow osteoarthritis may be noticed in neglected chondromatosis cases. Recurrences are common. Malignant transformation to synovial chondrosarcoma is a very rare but devastating complication associated with chondromatosis.