AIM: To investigate the clinical signs of blepharokeratoconjunctivitis (BKC) and evaluate the efficacy of penetrating keratoplasty (PKP) for the disease. METHODS: Sixteen patients (16 eyes) with BKC complicated by corneal perforation hospitalised at Shandong Eye Hospital were retrospectively analyzed. All patients received PKP. Participants were assessed for symptoms, clinical manifestations, the activity and damage grading of BKC. A paired t-test was used to compare the uncorrected visual acuity (UCVA) before and after surgery for the perforated eye. RESULTS: The mean age of the patients was 16.3y. Blurred vision is the most common discomfort, followed by redness, and then photophobia. The duration of ocular discomfort lasted for 3.2y, on average. Three (18.8%) participants were associated with rosacea, while 11 (68.8%) patients had recurrent chalazion or hordeolum. Demodex in eyelash follicles was positive in 11 (68.8%) cases. All corneal perforations were ≤3.0 mm in diameter. The perforation was located mainly in the inferior cornea (68.8%). The mean area of corneal vascularisation was 3.0 quadrants. All patients manifested bilateral BKC, with the perforated eyes ranked as severely damaged and presenting with severe inflammation. Most contralateral eyes manifested mild damage with no active inflammation. Majority (68.8%) of the perforated eyes were treated with PKP using a minimal graft. The UCVA increased significantly at the final follow-up (mean, 21mo; P<0.001), with the manifestation of BKC alleviated greatly. None of the patients developed immune rejection or other serious complications. CONCLUSION: BKC combined with corneal perforation occurs mainly among young people with a long history of ocular discomfort. PKP, especially using a minimal graft, is an effective and safe option for treating the disease.