Thalassemia adalah penyakit kronik yang menyebabkan penurunan kadar hemoglobin karena gangguan sintesis hemoglobin akibat mutasi satu atau lebih gen globin. Thalassemia dapat menyebabkan gangguan status gizi. Status gizi penderita thalassemia dipengaruhi oleh keadaan anemia kronik, kelebihan zat besi akibat kepatuhan minum obat kelasi besi yang rendah, usia saat terdiagnosis, kadar hemoglobin saat akan dilakukan transfusi, nutrisi, dan penyakit penyerta. Tujuan penelitian ini mengetahui hubungan antara kadar hemoglobin dan status gizi pada penderita beta-thalassemia major di RSUD Al-Ihsan Provinsi Jawa Barat. Penelitian ini menggunakan metode analitik dengan melihat data rekam medis pasien beta-thalassemia major di Klinik Anak RSUD Al-Ihsan periode Maret–Juni 2020 dengan rancangan studi cross-sectional. Kriteria inklusi: usia 0–18 tahun, terdapat data berat badan, tinggi badan, dan kadar hemoglobin. Dari 92 anak yang menderita beta-thalassemia major terdapat 82 anak yang memenuhi kriteria inklusi, kebanyakan berusia 1–5 tahun (40%) dengan jenis kelamin laki-laki 43 anak dan perempuan 39 anak. Pada anak usia ≤5 tahun: 85% status gizinya normal, gizi kurang 6%, perawakan normal 67%, perawakan pendek 18%, dan perawakan sangat pendek 15%. Anak usia >5 tahun: 71% status gizinya normal, gizi kurus 10%, sangat kurus hanya 2%, perawakan normal 35%, perawakan pendek 43%, dan perawakan sangat pendek 22%. Nilai p BB/TB pada anak ≤5 tahun adalah 0,494 dan TB/U 0,331. Pada anak usia >5 tahun didapatkan nilai p IMT/U 0,595 dan TB/U 0,230. Simpulan penelitian adalah kadar hemoglobin tidak memiliki hubungan dengan status gizi pasien beta-thalassemia major. THE RELATIONSHIP BETWEEN HEMOGLOBIN LEVELS AND NUTRITIONAL STATUS IN Β-THALASSEMIA MAJOR PATIENTS IN AL-IHSAN REGIONAL GENERAL HOSPITAL WEST JAVA PROVINCEThalassemia is a chronic disease that causes a decrease in hemoglobin level due to hemoglobin synthesis disorders due to mutations in one or more globin genes. Thalassemia can cause nutritional status disorders. Factors that influence nutritional status are age at diagnosis, hemoglobin level at the time of transfusion, chronic anemia, iron overload due to low adherence to taking iron-chelating agent drugs, nutrition, and comorbidities. This study aims to determine the relationship between hemoglobin levels and nutritional status in beta-thalassemia major patients in Al-Ihsan Regional General Hospital West Java province. This study used an analytic method by looking at the medical record data of beta-thalassemia major patients at the Children’s Clinic of Al-Ihsan Regional General Hospital during March–June 2020 with a cross-sectional study. The inclusion criteria were children aged 0–18 years, and there were data on body weight, body height, and hemoglobin level. Of the 92 beta-thalassemia major patients, 82 met the inclusion criteria, mostly aged 1–5 years (40%) with male 43 children and female 39 children. Children aged ≤5-years: 85% normal nutritional status, 6% wasted, 67% normal stature, 18% stunted, and 15% severely stunted. Children >5 years aged: 71% normal nutritional status, 10% wasted, 2% severely wasted, 35% normal stature, 43% stunted, and 22% severely stunted. On children aged ≤5-years, a p value BW/BH was 0.494, and a p value BH/A was 0.331. On children aged >5-years, a p value BMI/A was 0.595, and p value BH/A was 0.230. The conclusion is there is no relationship between hemoglobin levels and nutritional status in beta-thalassemia major patients.