Abstract: Objective A patient with fructose intolerance (HFI) with ALDOB compound heterozygous mutations was identified, and the follow-up data for 30 years was summarized. Methods The clinical manifestation and the experience of diet control were retrospectively summarized, and the gene sequencing results and the long-term outcome were also reported. The recipes that could cause HFI symptoms in the patient were systematically selected and recorded. The clinical information of patients with the known pathogenic mutations of ALDOB gene in HGMD was collected through literature review, the potential relationship between diet control and follow-up outcome was analyzed in patients with detailed clinical information. Results A 4-year-old girl suffered from fever due to unknown reasons, and the symptoms were relieved after vomiting. From the 20th day after birth, vomiting was observed immediately after the milk feeding. After changing to rice porridge, vomiting symptom was alleviated. In the childhood, due to the observation that the patient would vomit after eating foods like watermelon and cakes, the intake of sugary foods was avoided. At the age of 7 years, the patient was diagnosed as "developmental delay, inherited metabolic disorder？" with the chief complaints of low-fever, hunger after eating sweet foods and vomiting could relieve the symptoms. Meanwhile, parents started to select and record fruits, vegetables and other foods that could cause vomiting. The patient was followed up to 30 years old, with the height of 174.6 cm, the weight of 57.6 kg, normal intellectual development. No abnormalities were observed in heart, lung, thyroid, liver and kidney functions. The patient summarized detailed dietary contraindications and expected to perform genetic testing to identify the inherited metabolic diseases for diagnosis. The patient was tested by whole genome sequencing and Sanger validation and it turned out that she carried compound heterozygous mutations of ALDOB gene ［NM_000035, exon4:c.360_363delCAAA(p.N120Kfs*32); exon9:c.1013C>T (p.A338V)］. A338V was inherited from her father and N120Kfs*32 from her mother. In this article, 33 known pathogenic mutations of ALDOB gene reported by HGMD with detailed clinical information were collected. Six of the 8 (75%) patients without diet control had poorer prognosis than those with diet control (9/18, 50%). Among the diet-controlled patients, 55.6% patients of poor prognosis were with frameshift mutations, higher than the percentage of patients with good prognosis (27.8%). Conclusion Diet control is important for improving prognosis. However, there is a risk of poor prognosis for patients with frameshift variants even with well diet control, which need stricter diet control and close follow-up.