This case is notable for its unusual presentation in the external jugular vein, as glomeruloid hemangiomas have been infrequently reported and are mostly associated with systemic conditions such as POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin changes) or in cutaneous manifestations (4, 5). The gene discussed is MYOM2; the disease is POEMS syndrome.