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Pyoderma gangrenosum (PG) is a destructive, necrotizing, noninfective ulceration of the skin. Periorbital PG is extremely rare, and may progress, with eventual loss of the eye. We report a 47-year-old woman who presented with a right periorbital swelling of 8 days duration and concurrent acute rhinosinusitis. A clinical diagnosis of right periorbital PG was made, and treatment instituted with intravenous methylprednisolone and antibiotics, resulting in rapid resolution of the condition. The clinical features in this case, together with the past history of histologically confirmed PG, enabled a rapid diagnosis to be made, with early administration of treatment resulting in minimal scarring. The clinical picture could easily be confused with periorbital cellulitis, and this case demonstrates the importance of considering the differential diagnoses of periorbital swelling.