Aim and objectives: Thymic carcinoids are rare, incidentally detected in anterior mediastinal tumor, accounting for 2–4% of mediastinal tumor with an incidence of 0.18 per 1,000,000. Notably, 25% of thymic carcinoids are associated with MEN-1 syndrome. They are often detected incidentally, though some patients present with hormone-related symptoms, namely carcinoid syndrome, Cushing's syndrome, and very rarely acromegaly. We hereby present a case of thymic carcinoid, which was incidentally detected in a male patient who was evaluated for multiple head and neck AV-malformations. Case description: A 37-year-old male came with preauricular swelling. He had similar complaints of left preauricular swelling which was operated as parotid swelling, intraoperatively found to have AV malformation, and final histopathology reported as AV malformation. A few months later, he developed recurrent swelling was at the same site. On evaluation with MRI head and neck, along with multiple AV malformations, an anterior mediastinal mass was detected. On further evaluation, it was diagnosed as a thymic carcinoid with lymph node metastasis without distant metastasis. Functional and MEN1 syndromic evaluation were done, which were negative. The case was discussed in the tumor board meeting, and a decision was taken for surgical excision of the thymic tumor and observation for AV malformations. Sternotomy and Excision of the mass with lymph nodes are done in a cardiothoracic vascular operation theatre with the help of a cardiothoracic surgeon. Postoperatively, a patient recovered well. Histopathology revealed typical carcinoid (TC) with lymph node metastasis. The case was re-discussed in the tumor board meeting and planned for follow-up. Conclusion: Thymic carcinoids are rare mediastinal tumors that are incidentally detected, which mandates comprehensive functional, syndromic evaluation, and adequate surgical resection. Clinical significance: In our case, thymic carcinoid was indolent, typical, and found in association with multiple head and neck AV malformations. The association between these two is unknown. After a thorough search of the literature, this is probably the first case of the association of thymic carcinoid with multiple AV malformations.