Background Neurofibroma is a kind of benign neoplasm that derives from nervous tissues. Though this tumor is the most common types in the peripheral nervous system, it is rarely seen in the choroid and easy to be misdiagnosed of choroidal melanoma. The aim of this study was to review the clinical features of neurofibroma of the choroid in the Chinese race. Methods A retrospective case series design was used. Two male and one female patients diagnosed with choroidal neurofibroma in Beijing Tongren Eye Center were included in this study. The clinical histories were abstracted from the patients' medical records. Routine eye examinations including visual acuity, intraocular pressure, slit lamp and ophthalmoscope were performed. Auxiliary examinations included fluorescein fundus angiography (FFA), AB-ultrasound scan, color doppler imaging (CDI), and magnetic resonance imaging (MRI). Local resection of the tumors was performed and the specimens underwent pathological examinations. Results The tumors were of yellow-pink color with pigmentation on the surface. CDI showed arterial blood signals in the tumor and MRI showed high-intensity in the T1-weighted image and a slightly increased intensity in the T2-weighted image. FFA and indocyaninegreen angiography demonstrated the tumors were of hypofluorescence at early stage and hyperfluorescence with prominent leakage at late stage. The pathological examination confirmed the tumors were choroidal neurofibroma. After 5-10-year follow-up, there were no recurrences of the tumors and the retinas were well attached. Conclusions Choroidal neurofibroma is difficult to be diagnosed clinically and pathological confirmation is important. These tumors can be managed conservatively by local resection. Chin Med J 2012;125(10):1832-1835