Sclerema neonatorum (SN) is a rare condition with grave prognosis seen in premature neonates. It generally occurs in the first week of life and is associated with sepsis and hypothermia. The patient presents with rigid skin involving the whole body but sparing the palms, soles, and genitalia. The skin is cool, waxy, and has mottled discoloration. It may be associated with sepsis, hypothermia, hypocalcemia, and congenital anomalies. Death usually occurs due to septicemia in a majority of the cases. Maternal complications such as premature rupture of membranes, preeclampsia, eclampsia, and placenta previa may be associated. Treatment of SN is unsatisfactory, and it carries a bad prognosis. In the majority of patients, the outcome is fatal, and death results from sepsis. Early diagnosis of SN is of paramount importance, as early treatment of sepsis can reverse features of SN.