We report a rare case of anterior chamber live dirofilariasis presenting as anterior uveitis. A 60-year-old man presented with dimness of vision in the right eye for 1 month. Vision recorded was 6/18 P, N 18 in the right eye. Slit lamp examination of the right eye revealed anterior uveitis with a moving nemathelminthes. The worm was removed live from the anterior chamber under local anesthesia with assisted methyl cellulose delivery and post-operatively, the worm was examined directly under light microscope. Morphometric measurement showed length of the worm was 6.061 mm. A thin, pale, slender worm was diagnosed as immature female Dirofilaria repens and was documented completely. Patient had made an excellent recovery of vision and intraocular inflammation after the surgical removal of the worm. Intraocular infection of dirofilaria is a rare presentation and successful surgical removal of the worm resulted in complete recovery of uveitis and visual status in the affected eye.