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It is a rare anomaly that presents with various complications. A 14\u2010year\u2010old boy diagnosed with Ask\u2010Upmark kidney was referred for dental care. The patient presented with mouth breathing, unsatisfactory occlusion, and severe drug\u2010induced gingival hyperplasia, which hindered speech, diet, and oral hygiene. Imaging revealed the presence of all permanent teeth, but with delayed eruption, oblique eruption pathways, and risk of impaction. Management included oral hygiene instructions, prophylaxis, and ulectomy to facilitate tooth eruption. This case highlights the oral manifestations associated with the management of Ask\u2010Upmark kidney, specifically drug\u2010induced gingival enlargement and eruption disturbances. 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