The co-occurrence of inflammatory eye disease and hidradenitis suppurativa (HS) is uncommon; ocular involvement typically manifests as anterior uveitis. In this report, we present a unique case of peripheral ulcerative keratitis (PUK) where both infectious and rheumatological workup was negative, leading us to consider the possibility of an association between this condition and severe HS. As far as we are aware, there have only been six documented cases of PUK associated with HS.