Purpose: To report a child with Henoch-Schoenlein purpura associated with internuclear ophthalmoplegia. Methods: A case report. Results: An 11-year-old girl was admitted to our outpatient clinic with sudden diplopia associated with ataxia and fever. Ophthalmic examination revealed limitation of adduction in the left eye on attempted right gaze with a horizontal nystagmus in the right eye, indicating left internuclear ophthalmoplegia. Physical examination demonstrated that the child had pain and swelling of the left ankle and shoulders with diffuse palpable purpuric rash on her buttocks and lower extremities. Detailed laboratory evaluation was performed and the child was diagnosed as having Henoch-Schoenlein purpura. Intravenous prednisolone (2 mg/kg/day) was started and, after two months, diplopia, nystagmus, and the limitation of adduction disappeared with resolution of the disease. Conclusion: This case is the first report of internuclear ophthalmoplegia associated with Henoch-Schoenlein purpura, a well-known vasculitis. The vessels supplying the medial longitudinal fasciculus may be affected by the vasculitic process of the disease. Internuclear ophthalmoplegia may be a presenting symptom of this disease and ophthalmologists should be aware of it in the differential diagnosis of eye movement disorders.