A 43-year-old man came to our clinic suffering from a malodorous fistulating tract of the lower extremities and a purulent draining sinus of the groin embedded in keloidal tissue. He was diagnosed with Keratitis-ichthyosis-deafness (KID) syndrome via sequencing analysis 11 years prior. A punch biopsy of lesion on the lower extremities was done. Clinically and histologically, Hidradenitis suppurativa (HS) was diagnosed in association with KID syndrome. Previous therapies with antibiotics and isotretinoin did not produce satisfactory improvements. We started adalimumab with an induction dose of 160 mg followed by 80mg and a maintenance dose of 40 mg every weeks. After 8 weeks on adalimumab therapy, the lesion showed marked improvement. Modified Sartorius scores showed progressive improvement during treatment, from 43 at the first visit to 17 after 8 weeks of treatment with adalimumab. Hurley stage was also improved from 3 to 2. Despite appropriate treatment, HS recurs frequently. KID syndrome is a rare congenital disorder of the ectoderm that gives rise to keratitis, erythrokeratoderma and neurosensory deafness. In addition to xerosis, HS has rarely been reported as a skin symptom accompanying KID. Antibiotics, acitretin, retinoids and anti-TNF drugs have been reported to treat it, but cases of severe HS are usually refractory to treatment. Herein, we report a case of HS in association with KID syndrome treated with adalimumab.