The purpose of this paper was to analyse the causes, pathogenesis, diagnostic modalities and treatment outcomes of microsporidial keratoconjunctivitis (MKC). Microsporidia are increasingly recognized as opportunistic infectious pathogens in immunocompromized patients causing keratoconjunctivitis. In the recent years, there has been a surge in reports of MKC in immunocompetent individuals presenting with stromal keratitis. A detailed literature search was done using Medline, OVID, Cochrane Library, UptoDate and Google Scholar databases with the terms microsporidia, keratitis, conjunctivitis, immunocompromized and immunocompetent. The articles were reviewed to determine the spectrum of clinical presentation, disease course, investigations, treatment modalities and outcome. Thirty-six publications were reviewed, and 151 cases of MKC were included for this review. The main presenting features included pain, redness, photophobia, epiphora and blurring of vision. Duration of the symptoms lasted between 4 days and 18 months. Light microscopy with modified trichrome stain was most commonly used to diagnose MKC. Resolution of symptoms was most commonly achieved with oral albendazole and/or topical fumidil B. Topical fluoroquinolones are also effective as a monotherapy as suggested by recent studies. Clinical outcome was good (visual acuity = 6/12) for the patients who presented earlier (=1 month) (75% of cases with documented final best-corrected visual acuity). MKC occurs more commonly in immunocompetent individuals than expected and can be diagnosed in earlier stages. From our review, we conclude that the patients, who were diagnosed early and treated, had complete resolution of symptoms with a better clinical outcome.