To report a case of Niemann–Pick disease Type C (NP‑C) associated with Fuchs Heterochromic Iridocyclitis (FHI).

A 26 years old female with the complaint of decrease vision and flouter was referred to ophthalmic ward. According to her mother’s history, the patient had a medical history of developmental delay, auditory impairment,the history of generalized tonic–clonic seizures, and also ataxic gait since the toddler age and she had some school difficulties since childhood. Her ocular examination finding included of: diffuse fine keratic precipitates, mild iris atrophy, mild posterior subcapsular cataract, and mild (1+) vitreous cells in the right eye. The examination of the left eye was normal.

Here, a case of NP‑C disease who suffered also from FHI is presented.

NP‑C disease could be added to the long list of FHI associations.