Though majority of genetic analyzes in affected individuals and transgenic mice have found mutations in gene for loricrin as the underlying defect, a recent report demonstrated connexin mutations in a PSEK patient.[Loricrin is the major structural component of the cornified cell envelope while connexins are the protein units of gap junctions distributed in skin, nervous system, internal ear, cornea, and lens. Due to unavailability of genetic testing in our institute, exact mutation in our patient could not be elucidated. Associated neurological abnormalities have been described recently.We hypothesize that unusual association of ocular and neurological complaints in our patient could be due to underlying mutation of gap junction protein, connexin.clinically characterized by well-demarcated erythematous and hyperkeratotic plaques that are distributed with an almost perfect symmetry on the head, extremities, and buttocks. Onset is usually in early childhood, which progresses over the next few years and then remains stable over time with morphology, color, and site remaining constant. Rarely, they may partially regress after puberty. Patients are usually mentally and physically unaffected. Differential diagnoses include EKV, psoriasis, and pityriasis rubra pilaris