The objective of this study was to assess outcomes of hypoplastic left heart syndrome (HLHS) patients weighing ≤2.5 kg throughout staged palliation. We performed a single-center retrospective review. Abstracted data included gestational age, birth weight, presence of noncardiac anomalies, and survival through Fontan. Fifty-two patients met inclusion criteria, with a median birth weight of 2.14 kg and gestational age of 36 weeks. Five patients received comfort care only. Of 47 patients who underwent initial surgical palliation, 51% survived to initial hospital discharge. Birth weight and gestational age (GA) were similar between survivors and nonsurvivors. Compared with survivors, risk factors for death prior to initial hospital discharge were as follows: small for GA (P = 0.005), noncardiac anomalies (P = 0.04), need for post-perative extracorporeal membrane oxygenation (P = 0.0004), and conversion from initial palliation to Sano shunt (n = 5, no survivors). Operative survival following Stage 2 palliation was 91% (21/23) and 94% after Fontan (17/18). Overall survival for palliated patients from birth through Fontan was 36%. Low-birth-weight neonates with HLHS have poor overall survival through the Fontan operation, with highest mortality following Stage 1 palliation. Being small for GA and the presence of noncardiac anomalies are important preoperative risk factors for early mortality.