The authors describe four children in whom idiopathic orbital pseudotumor (IOP) was the initial solitary finding with systemic inflammatory disease developing later. Four children were seen over a five-year period. Mean age was 9.75 years (2-14). Three were white and one Hispanic, three were female, IOP was in the right eye for three and bilateral for one. The lesion involved the lacrimal gland and orbital soft tissue in all with extraocular muscle involvement also in three cases. Management included oral prednisolone and two with excisional biopsy. Systemic inflammatory disease developed in the ensuing months for all cases including panciarticular juvenile idiopathic arthritis, eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis and Crohn’s disease. Early diagnosis of systemic disease may have been possible with systemic screening at presentation. However, the authors acknowledge that full systemic evaluation may not be warranted for all cases. Close observation and suspicion for systemic inflammatory disease is reasonable for children presenting with IOP.