Ocular microsporidiosis comprises two entirely different spectra of disease as keratoconjunctivitis and stromal keratitis. Microsporidial keratoconjunctivitis (MKC) has been increasingly reported in the past two decades, probably due to raised awareness, simpler diagnostic procedures, and a better understanding of the clinical presentation. It is characterized by the presence of raised, coarse, punctate, multifocal, round to oval, greyish-white corneal epithelial lesions which usually evolve into nummular scars before resolution. Conjunctivitis seen is non-purulent and of mild-moderate intensity, with mixed papillary-follicular reaction. The mode of transmission and pathogenesis is poorly understood. Despite lack of inflammatory response, uncommon associations reported were- endotheliitis, corneal edema, limbitis, uveitis, and sub-epithelial infiltrates. There has been no consensus on the management of MKC. It varies from the use of multiple antimicrobial agents to simple lubricants. The majority of the disease goes underdiagnosed or misdiagnosed and treated as adenoviral keratoconjunctivitis, with topical steroids or anti-virals empirically. Changing trends have been noticed in the pattern of infection, possibly with increasing evidence of Vittaforma corneae as causative organisms, previously reported to cause stromal keratitis. An elaborate review of the past and present literature on MKC is provided in this review article, along with gaps in knowledge, and future directions of research.