Renal tubular acidosis (RTA) encompasses a group of disorders characterized by impaired kidney acid-base regulation, resulting in metabolic acidosis. Nephrolithiasis, the formation of kidney stones, represents a significant complication of RTA. Despite its clinical importance, the relationship between RTA and nephrolithiasis remains complex and poorly understood. This review provides a comprehensive overview of nephrolithiasis in RTA, focusing on its pathophysiology, diagnostic challenges, and management strategies. Mechanisms underlying kidney stone formation in RTA, including altered urinary pH, electrolyte abnormalities, and mineral metabolism disturbances, are explored in detail. Clinical manifestations and diagnostic considerations specific to nephrolithiasis in RTA patients are discussed, emphasizing the importance of a comprehensive metabolic evaluation and imaging procedures for accurate diagnosis. Current therapeutic approaches for treating kidney stones in RTA patients, including dietary modifications, pharmacological interventions, and surgical interventions, are reviewed. By elucidating the intricate interplay between RTA and nephrolithiasis, this review aims to enhance clinical understanding and guide optimal management strategies for patients with this challenging renal disorder.