Enamel renal syndrome is a unique syndrome associated with kidney agenesis associated with kidney agenesis, amelogenesis imperfecta, and gingival hyperplasia. The prevalence rate of this rare syndrome is <1/1,000,000. A 17-year-old male patient came to the department of periodontics, with a chief complaint of dislodged crown in the anterior teeth region. On clinical examination, the patient had teeth with mottled enamel and gingival enlargement. The orthopantomograph and gingival biopsy revealed pulpal calcification and gingival calcification, respectively. Furthermore, the renal ultrasonography revealed absence/agenesis of the left kidney. Thus, based on radiographical, histological, and ultrasound investigations, the patient was diagnosed with nephrocalcinosis syndrome. The patient was treated with periodontal therapy and prosthodontic full-mouth rehabilitation. This case report highlights the need of a periodontist to be acquainted about the signs and symptoms of the syndrome to benefit an individual in the right diagnosis and treatment plan.