A 2-year-old boy presented with a 3-month history of nontender masses involving the right superotemporal orbit and scalp. Over the three-week period before presentation he also developed nodules on his wrist, abdomen, and buttocks. The remainder of the ocular and general physical exam was unremarkable. A biopsy of the right orbital mass demonstrated stellate areas of collagen necrosis surrounded by pallisading epithelioid histiocytes and scattered multinucleated giant cells. Special stains for connective tissue mucin were positive in the areas of necrosis. The diagnosis reached pathologically and clinically were that of pseudorheumatoid nodules and granuloma annulare syndrome respectively. These two diagnostic terms have been used independently in the ophthalmologic and dermatologic literature, despite the identical histologic and clinical behaviors seen by both medical groups. A unified nomenclature system of pseudorheumatoid nodule for the histopathology of an individual lesion and granuloma annulare for the syndrome is suggested.