We report a case of a 2-week-old white female who presented with large stellate atrophic skin defects on bilateral thighs and knees at birth. The pregnancy was complicated by the death of monozygotic twin at the 16th week of gestation. This represents aplasia cutis congenita with fetus papyraceus. This rare condition is defined as the congenital skin defect and intrauterine death of fetus with or without a stillborn fetus pressed flat by the growing twin (fetus papyraceus) at delivery time. Aplasia cutis congenita coexisting with fetus papyraceus has a distinctive and reproducible distribution pattern of bilateral symmetrical truncal, buttock, and thigh lesions related to fetal death during the late first to early second trimester. There are at least 44 reported cases of this disorder in the English literature. We discuss our case and review the literature.