Abstract Background : Split hand and foot malformations (SHFMs) are found in a large heterogeneous group of disorders, resulting from maldevelopment of the central rays of the limb buds, and are reported to occur in one in 10 000 to 90 000 live births. They can occur as an isolated defect or as a part of a variety of syndromes. The syndromic form, with associated anamolies such as tibial aplasia, mental retardation, ectodermal and craniofacial findings, orofacial clefting and deafness. Case : A 5-year old boy presented with deformed hands and feet since birth. There were median clefts of both hands and feet. There were no other dysmorphic features. He was born at a gestational age of 39 weeks by Caesarean section and weighted 3,960g. He is a multicultural family. His father is Korean and his mother is Vietnamese. His mother had a thyroid disease and was able to take medication. He has a lobster-claw hand on both side, and cleft foot with three central ray deficiency (type IV) on both side and 1st and 5th toe varus deformity. His father also has limb malformations. He received correction of cleft foot by simple closure on both side in 2016. He lost his hearing and underwent cochlear implant surgery three years ago. He also showed delayed language development on language tests. Genetic testing was not performed due to personal circumstances. He came to our hospital because of the seizure like activity which occurred in September 2016. The seizure persisted for 3 minutes with generalized tonic clonic seizure, and there were no other associated symptoms such as fever. Electroencephalography was performed and its findings show us depressed wave on right mid-posterior area and rare sharp waves were found on both frontocentral area. In brain MRI, Subependymal heterotopia was found in the Rt occipital horn. He was treated by levetiracetam 40mg/kg/day, his symptoms were improved and discharged. Conclusion : The syndromic form of SHFM expressed in some patients is associated with anomalies asintellectual disability in 33%, ectodermal and craniofacial findings in ＞35% and orofacial clefting, and neurosensory hearing loss in ＞35%. We report a case of syndromic form SHFMs patients with subependymal heterotopia.