Abstract This report describes a rare case of ophthalmic dirofilariasis in a 2-year-old boy with redness, irritation, pain and foreign body sensation in the right eye. Slit lamp examination demonstrated a thread-like whitish nematode in the anterior chamber of the right eye that twisted around itself. The nematode worm (35 mm long and 150–200 μm width) was removed surgically. The presence of the smooth cuticular surface without longitudinal ridges and the vulva showed that it could be a female Diroflaria immitis . PCR amplification was done to verify the Diroflaria species. PCR amplification and sequence analysis of mitochondrial 12S rDNA confirmed that recovered worm was D. immitis . Ocular dirofilariasis caused by D. immitis is very rare, but it must be considered in humans living in endemic areas.