Angiocentric lesions of the head and neck encompass a variety of benign and malignant lesions. Not unexpectedly the sequelae of an angiocentric process independent of its benign or malignant nature is one of tissue ischemia with a potential for either breakdown or reparative fibrosis. Therefore, the clinical presentations can be very similar despite a varied pathogenesis. Among the benign reactive infiltrates that will be considered are angiocentric eosinophilic fibrosis, Wegener's granulomatosis, microscopic polyangiitis and cocaine associated mid line facial destruction. We will discuss other conditions which enter into the differential diagnosis either clinically or histologically including Erdheim Chester disease and mid line facial undermining unrelated to an angiocentric event specifically in the context of trigeminal trophic ulcer and relapsing polychondritis. The two main neoplastic conditions exhibiting angiocentricity are in the context of lymphomatoid granulomatosis and NK/T cell lymphoma; hence these two particular hematologic dyscrasias will be discussed in some detail in this review.