Ocular involvement of lymphoma may present as a primary orbital or intraocular lymphoma or as a manifestation of metastatic disease. Involvement of various ocular structures may be difficult to diagnose due to its rarity and nonspecific clinical presentation. Primary high-grade B-cell lymphoma with MYC and BCL2 rearrangements of the orbit has rarely been reported in the adult population and has not previously been reported in the pediatric population. Herein the authors present a unique case of orbital high-grade B-cell lymphoma in a pediatric patient, initially diagnosed as preseptal cellulitis. Careful observation and escalation of care ultimately led to the accurate diagnosis and complete remission of the disease.
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