A retrospective review identified 41 patients with angiodysplastic lesions of the extremities. Twenty-two patients had a mass, 11 had limb length discrepancy and/or hemihypertrophy, and two had recurrent joint effusions. Enhanced computed tomography (CT) and magnetic resonance imaging (MRI) were most valuable for both diagnosis and preoperative planning. Many patients were diagnosed clinically and treated symptomatically. Twenty-nine patients (71%) required operation. Descriptive pathology and histologic diagnosis were not determinants of clinical outcome, whereas anatomic location and overall size were predictive of symptomatology. Subcutaneous hemangiomas irritated sensory nerves, intramuscular lesions mimicked compartment syndromes, intraarticular lesions caused recurrent hemarthroses, and periarticular or large lesions resulted in hypertrophy or limb length discrepancy. Simple excision under tourniquet control with incomplete exsanguination is possible but often incomplete (48% recurrence) owing to the invasive nature of the lesion.