Myopia is a prevalent condition among Asians. Orthokeratology lens has gained popularity as a method of myopia control. This systematic review is to summarize the clinical profile of infectious keratitis in association with orthokeratology lens wear. We searched in the PubMed and EMBASE for articles adopting the search strategy "(orthokeratology lens OR orthokeratology) AND (bacterial eye infection OR keratitis OR cornea ulcer OR microbial keratitis OR bacterial keratitis)", from the start date of the databases to August 23, 2016. Articles reporting infectious keratitis in orthokeratology lens users with data of individual cases were considered eligible for this systematic review. We recorded the outcome measures including method of diagnosis, etiological agents, duration and mode of treatment and treatment outcomes. Our literature search yielded 172 papers. After removing duplicated and irrelevant reports, we included 29 articles for data analysis, involving 173 eyes. Among all reported cases, the mean age at presentation was 15.4±6.2years, with a female preponderance (male-to-female ratio 1:1.7). Positive microbiological cultures were reported in 69.4% of cases, with Pseudomonas aeruginosa and Acanthamoeba being the most common etiological agents. The mean duration of hospitalization was 7.7±6.7days. Mean LogMAR visual acuity at presentation was 1.17±0.78, increased to 0.33±0.41 at final visit (p<0.001). Despite early intervention and treatment, the majority of infections resulted in the formation of corneal scars and almost 10% of eyes needed surgical treatment. Timely awareness and treatment of keratitis should be emphasized to the users.