In conclusion, we described in detail the prenatal clinical features of a Chinese pedigree with LCCS3 caused by biallelic pathogenic variants in PIP5K1C. The identification of the novel variant and novel phenotypes expands the variant spectrum of PIP5K1C and enriches the clinical characteristics of LCCS3, which will be valuable for prenatal diagnosis and genetic counseling. Here, PIP5K1C is linked to lethal congenital contracture syndrome 3.