Extralymphatic filariasis caused by filaria of zoonotic origins has been frequently reported in Thailand over recent years. Here, we report the first case of ocular filariasis in a 7.5-year-old Thai boy who initially presented with progressive conjunctival redness and blurred vision in his right eye. A small, slender, coiled worm was found and surgically removed from the right anterior chamber. Histopathological examination illustrated predominant eosinophilic inflammation surrounding the parasite, which showed smooth and thin cuticle, prominent lateral chords, flat and broad muscle cells, one intestine, and two reproductive tubes with unsegmented ova, typically characteristic of a female adult Brugia filarial nematode. The parasite was also molecularly identified as B. pahangi, based on mitochondrial cytochrome c oxidase subunit I sequence analysis. The patient was then empirically prescribed albendazole, systemic prednisolone, and topical methylprednisolone. Unfortunately, his vision did not recover after 2 months due to severe maculopathy, most likely resulting from parasitic infestation and subsequent vitreous inflammation. To the best of our knowledge, this is the first case of ocular infestation by B. pahangi with visual complications that occurred outside a filariasis-endemic area of Thailand. Furthermore, this report provides clinical data on preceding cases of B. pahangi filariasis formally reported in southeast Asian countries, including Thailand and Malaysia, which facilitate a better understanding of the epidemiology of this sporadic zoonotic infection for effective disease elimination.