A 56-year-old woman with sickle cell anemia (SCA) and metastatic neuroendocrine tumor refractory to somatostatin analog received 4 cycles of peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE. The treatment schedule was delayed by multiple episodes of hospitalizations related to underlying SCA. PRRT was well-tolerated with stable renal, hematologic, and liver lab parameters. After PRRT, the disease remained stable for up to 13 months, and serum chromogranin levels declined by 45%. PRRT with 177Lu-DOTATATE can be safely administered for metastatic neuroendocrine tumors in patients with SCA with good anti-tumor effect. Disruption to the therapy schedule should be anticipated, given the potential for recurrent hospitalization for illnesses related to the underlying SCA.