Pythium keratitis is one of the chief etiologies of atypical microbial keratitis. It has an extremely grave prognosis. A low index of suspicion, its resemblance to fungal keratitis, and inappropriate diagnosis and management all contribute to the poor outcomes. The absence of adequate literature recommending a standard regimen further worsens the picture. Although Pythium resembles a fungus, it lacks ergosterol. Hence, antifungals are ineffective. While its successful management using antibiotics has been described, some conclude that surgery is the mainstay and recommend techniques to minimize recurrences. This review article aims to bring together the various modalities of diagnosis and therapy of Pythium keratitis. It focuses on the clues to clinical suspicion which would facilitate a prompt diagnosis. It also highlights the importance of high index of suspicion in presumed fungal keratitis with a poor response to antifungals. A search was carried out using PubMed and Google Scholar for articles in English language using the keywords: Pythium insidiosum, Pythium keratitis, management of Pythium keratitis, investigations in Pythium keratitis, Polymerase Chain Reaction in Pythium keratitis, diagnosis of Pythium keratitis, and treatment of Pythium keratitis. Articles were included if they were of high significance for this article. Case reports also were taken into consideration if they conveyed data of importance.