Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is rare and poorly understood and can affect a wide range of organ systems and is progressive in nature. Most commonly affecting the respiratory tract and kidneys, it has also been cited to affect the central nervous system (CNS) and skin. Proper and timely diagnosis will warrant appropriate treatment with slowing of disease progression. Our case illustrates a rare form of GPA with CNS and skin involvement that urges a wide differential for proper diagnosis.

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