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Glomus tumors are perivascular neoplasms, derived from modified smooth muscle cells of a neuromyoarterial glomuscommonly termed a glomus body-with the function of temperature regulation through arteriovenous shunting of blood (1). Glomus tumors are rare and have been reported in approximately 1.6% of patients with primary soft tissue tumors of upper and lower extremities (2). The lesion characteristically occurs in a digital subungual location, presenting with welllocalized pain that is exacerbated by temperature changes. Although tumors are most often located on the digits, up to 45% of glomus tumors are extradigital, with common locations being the hands, arms, legs, and trunk (3, 4). Glomus tumors may also develop in sites where normal glomus bodies may be sparse or even absent, such as the gastrointestinal tract, liver, lung, and trachea, as well as intraosseous and intraneural locations (3). In the musculoskeletal system, extradigital glomus tumors have been described around the shoulder, elbow, wrist, hip, knee, and ankle (5). There have been several reported cases of extradigital glomus tumors in the thigh. In the majority of these cases, the tumors were located superficially, such as the subcutaneous and intramuscular tissues (6). To our knowledge, there have been only three reported cases of periosteal glomus tumors adjacent to the femoral cortex (7-9). We report a rare case of a histologically proven glomus tumor found in the periosteal location of the distal femur. An Unusual Cause of Knee Pain: Periosteal Glomus Tumor of the Distal Femur    :