A 66-year-old female presented with a 6-month history of increasing right-sided facial pain and diplopia. Right ocular movements were restricted in adduction and downgaze. Superior oblique (SO) enlargement was seen on an initial MRI scan, and subsequent diffuse infiltration was seen on a repeat scan. An elevated cANCA and biopsy of SO confirmed Wegener's granulomatosis (WG). We report a case of SO myositis as a predominant early feature of WG granulomatosis and review the literature regarding extraocular muscle involvement in WG. To our knowledge, early SO involvement has not been reported.