Since its description 50 years ago, pyoderma gangrenosum has continued to capture the attention and imagination of all those who see its dramatic presentation. Clinical observation still provides the only reliable diagnosis. As investigative techniques increase, more and more intriguing immunologic abnormalities associated with this disorder are discovered, but understanding of the pathogenesis remains elusive. It is now recognized as an independent condition as well as a co-condition with many systemic disorders. Many new treatment options are available, allowing much individualization of treatment. For now, pyoderma gangrenosum remains an impressive, relatively easily recognized, but poorly understood disease.