Purpose: To describe the first case of interstitial keratitis (IK) secondary to Anaplasma phagocytophilum infection. Methods: We present a case of IK secondary to A. phagocytophilum infection diagnosed using nested polymerase chain reaction. Results: A 32-year-old man with an unremarkable medical history presented with a 6-month history of bilateral decreased vision and ocular redness associated with a progressively enlarging white spot in the left eye. The patient was diagnosed with bilateral IK, and a thorough systemic workup was performed. The final diagnosis of A. phagocytophilum was made based on whole-blood Nested-PCR. Doxycycline was initiated with 100-mg BID tablets for 3 weeks. On completion of oral treatment, a left penetrating keratoplasty was performed. The excised cornea showed infiltration of inflammatory cells and scar tissue as well as superficial and deep stromal neovascularization. Conclusions: A. phagocytophilum is an underdiagnosed entity, and IK may be associated with its clinical manifestations. We hope that this illustration of a rather atypical finding adds to the clinical spectrum of ophthalmic conditions that can be observed in association with the disease.