Rationale: Cardiac primary spindle cell sarcoma is 1 of the rarest cardiac malignancies, with only a few cases reported so far. Herein, we reported a case of left atrial spindle cell sarcoma diagnosed and treated by a multidisciplinary approach, and retrospectively reviewed other reported cases. Patient concerns: A 49-year-old woman presented to our hospital with 2 weeks of gradual onset of dyspnea on exertion, dry cough and subacute fever. Diagnosis: The patient was initially revealed a left atrium mass by 2-dimensional transthoracic echocardiography. Based on the contrast-enhanced echocardiography and cardiac magnetic resonance imaging, she was subsequently suggested to have a cardiac malignant tumor. And the post-operative histopathology confirmed the tumor to be a cardiac primary spindle cell sarcoma. Interventions: The tumor was completely resected using autotransplantation. The patient was referred for polychemotherapy afterwards. Outcomes: Our patient underwent the tumor resection, with subsequent adjuvant polychemotherapy, and the tumor has not recurred during 12 months of follow-up. Lessons: Due to the rarity of these tumors and nonspecific symptoms, they are often difficult to diagnose preoperatively and missed occasionally. Thus, improving our understanding of the disease and facilitating its early diagnosis are essential.