ALK fusion genes are diverse. Approximately 30 different ALK fusion protein partners have been described previously, and some of these fusion proteins have been reported to be effective against ALK-tyrosine kinase inhibitor (TKI). ALK rearrangements often occur at a common breakpoint in exon 20 of the genome. SLC8A1-ALK, a novel fusion protein partner, comes from exon 2 of the SLC8A1 gene rearranged with exon 20 of the ALK gene. Here, we reported a patient with advanced lung adenocarcinoma harboring a SLC8A1-ALK fusion who benefited from first-line treatment with alectinib. After 2 months of taking alectinib, the targeted lung lesions and intrahepatic metastases regressed significantly. To date, the patient has achieved nearly 1 year of progression-free survival while taking the drug. Given the diversity of ALK fusion genes and the different efficacy of ALK-TKIs, we believe that this case report has an important clinical reference.